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1
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Wang H, Li Q, Han S, Tian H. Rare case report: primary small-cell neuroendocrine carcinoma of the gallbladder. Front Oncol 2025; 15:1524974. [PMID: 40248205 PMCID: PMC12003105 DOI: 10.3389/fonc.2025.1524974] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/08/2024] [Accepted: 03/03/2025] [Indexed: 04/19/2025] Open
Abstract
Gallbladder cancer (GBC) accounts for 1.7% of all cancer-related deaths. Neuroendocrine carcinoma of the gallbladder (GB-NEC) is a rare subtype of GBC that is more malignant than GBC. Small-cell neuroendocrine carcinoma of the gallbladder (GB-SCNEC) is a rare malignant tumor with a low incidence. To date, no universally accepted or satisfactory treatment exists. This case report details the clinical presentation, diagnostic process, and treatment strategy of a patient with GB-SCNEC. The analysis of this rare case is intended to provide clinicians with diagnostic and therapeutic insights for future research.
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Affiliation(s)
| | | | | | - Hu Tian
- Department of General Surgery, The First Affiliated Hospital of Shandong First Medical University & Shandong Provincial Qianfoshan Hospital, Jinan, China
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Li C, Lv P, Yanyan L, Yin M, Li H. Immune checkpoint inhibitor therapy for primary neuroendocrine carcinoma of the gallbladder: A case report and literature review. Medicine (Baltimore) 2024; 103:e40178. [PMID: 39470513 PMCID: PMC11521044 DOI: 10.1097/md.0000000000040178] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/07/2024] [Accepted: 10/03/2024] [Indexed: 10/30/2024] Open
Abstract
RATIONALE Gallbladder neuroendocrine carcinoma (GNEC) is indeed a relatively rare malignant tumor of the gallbladder with neuroendocrine differentiation and the ability to produce and secrete a number of neurotransmitters and hormones, characteristics that make its clinical presentation and biological behavior likely to be different from those of other types of gallbladder cancer. Current treatment mostly relies on surgery and adjuvant chemotherapy and radiotherapy. PATIENT CONCERNS We report a 53-year-old middle-aged male patient who underwent radical surgery for gallbladder malignancy after a diagnosis of neuroendocrine carcinoma of the gallbladder. DIAGNOSES Diagnosis of neuroendocrine carcinoma of the gallbladder based on the return of pathologic findings. INTERVENTION After local progression of postoperative chemotherapy with the first-line regimen of etoposide + cisplatin, an immune checkpoint inhibitor (traplizumab) + FOLIFIRI (fluorouracil + calcium folinate + irinotecan) regimen was used. OUTCOMES The patient achieved 20 months of clinical survival and ultimately died of myelosuppression. LESSONS The use of immune checkpoint inhibitors may become an effective tool in the treatment of neuroendocrine carcinoma of the gallbladder.
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Affiliation(s)
- Chao Li
- Hepatobiliary and Pancreatic Medicine Center, Weifang Peoples Hospital, Weifang, Shandong, P.R. China
| | - Pan Lv
- Hepatobiliary and Pancreatic Medicine Center, Weifang Peoples Hospital, Weifang, Shandong, P.R. China
| | - Liu Yanyan
- Hepatobiliary and Pancreatic Medicine Center, Weifang Peoples Hospital, Weifang, Shandong, P.R. China
| | - Maohui Yin
- Hepatobiliary and Pancreatic Medicine Center, Weifang Peoples Hospital, Weifang, Shandong, P.R. China
| | - Hao Li
- Hepatobiliary and Pancreatic Medicine Center, Weifang Peoples Hospital, Weifang, Shandong, P.R. China
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Chen L, Cao X, Luo X, Jiang T. Refractory pruritus caused by sintilimab and its clinical management: A case report. Heliyon 2024; 10:e34107. [PMID: 39100464 PMCID: PMC11295844 DOI: 10.1016/j.heliyon.2024.e34107] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/09/2023] [Revised: 07/03/2024] [Accepted: 07/03/2024] [Indexed: 08/06/2024] Open
Abstract
Several immune related adverse events (irAEs) were reported with the wide application of immune checkpoint inhibitors (ICIs) in tumors. ICI-related skin reactions are the most common, which are manifested as maculopapules, rash, pruritus, vitiligo, psoriasis, and lichenoid rash.Among them, the incidence of pruritus is second only to maculopapule/rash, but both often co-exist. The severity of pruritus is mostly mild to moderate and can be relieved after symptomatic treatment with antihistamines. Symptoms are slightly relieved after conventional treatment in patients with severe pruritus, but it easily recurs and eventually develops into refractory pruritus.The patient's quality of life may be affected and may also be life-threatening. We report a case of a patient with postoperative recurrence of gallbladder neuroendocrine carcinoma,who developed refractory pruritus after sintilimab use, which was relieved after naloxone infusion after unsuccessful conventional drug therapy. By analyzing the treatment plan of this typical case of immune-related refractory pruritus after using sintilimab, this report discusses how clinical pharmacists can provide individualized treatment of patients by using their expertise and clinicians' cooperation and complementation in treating clinically difficult cases. This case report may be used as a reference in treating patients with refractory pruritus after the clinical use of sintilimab.
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Affiliation(s)
- Li Chen
- Department of Pharmacy, Wusheng People's Hospital, 513 Jianshe North Road, Wusheng County, Guangan, 638400, Sichuan, China
| | - Xin Cao
- General Practice Department, Clinical Medical College and the First Affiliated Hospital of North Sichuan Medical College, 1 Maoyuan South Road, Nanchong, 637000, Sichuan, China
| | - Xing Luo
- Department of Oncology, Clinical Medical College and the First Affiliated Hospital of Chengdu Medical College, 278 Baoguang Street, Xindu District Chengdu, 610500, Sichuan, China
| | - Ting Jiang
- Department of Pharmacy, Clinical Medical College and the First Affiliated Hospital of Chengdu Medical College, 278 Baoguang Street, Xindu District Chengdu, 610500, Sichuan, China
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Liu F, Miao W, Nan J, Shi Z, Zhang A, Bo Y, Xu J. Clinical diagnosis and treatment of 37 cases of gallbladder neuroendocrine carcinoma. World J Surg Oncol 2024; 22:157. [PMID: 38877554 PMCID: PMC11177431 DOI: 10.1186/s12957-024-03436-z] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/23/2024] [Accepted: 06/01/2024] [Indexed: 06/16/2024] Open
Abstract
OBJECTIVE This study aims to investigate the clinical and pathological characteristics, treatment approaches, and prognosis of gallbladder neuroendocrine carcinoma (GB-NEC). METHODS Retrospective analysis was conducted on the clinical data of 37 patients with GB-NEC admitted to Shanxi Cancer Hospital from January 2010 to June 2023. The study included an examination of their general information, treatment regimens, and overall prognosis. RESULTS Twelve cases, either due to distant metastasis or other reasons, did not undergo surgical treatment and received palliative chemotherapy (Group 1). Two cases underwent simple cholecystectomy (Group 2); four patients underwent palliative tumor resection surgery (Group 3), and nineteen patients underwent radical resection surgery (Group 4). Among the 37 GB-NEC patients, the average pre-surgery CA19-9 level was 113.29 ± 138.45 U/mL, and the median overall survival time was 19 months (range 7.89-30.11 months). Of these, 28 cases (75.7%) received systemic treatment, 25 cases (67.6%) underwent surgical intervention, and 16 cases (64.0%) received postoperative adjuvant treatment, including combined radiochemotherapy or chemotherapy alone. The median overall survival time was 4 months (0.61-7.40 months) for Group 1 (n = 12), 8 months for Group 2 (n = 2), 21 months (14.67-43.33 months) for Group 3 (n = 4), and 19 months (range 7.89-30.11 months) for Group 4 (n = 19). A significant difference in median overall survival time was observed between Group 1 and Group 4 (P = 0.004). CONCLUSION Surgery remains the primary treatment for GB-NEC, with radical resection potentially offering greater benefits to patient survival compared to other therapeutic options. Postoperative adjuvant therapy has the potential to extend patient survival, although the overall prognosis remains challenging.
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Affiliation(s)
- Feng Liu
- First Clinical Medical School, Shanxi Medical University, Taiyuan, 030001, China
- Department of Head and Neck Surgery, Shanxi Provincial Cancer Hospital, Shanxi Hospital Cancer Hospital of Chinese Academy of Medical Sciences, Taiyuan, 030001, China
| | - Wentao Miao
- First Clinical Medical School, Shanxi Medical University, Taiyuan, 030001, China
| | - Jiang Nan
- Department of Plastic Surgery, Taiyuan Maternity and Child Health Care Hospital, Taiyuan Children's Hospital, Taiyuan, 030001, China
| | - Zhiyong Shi
- Department of Hepatobiliary and Pancreatic Surgery, Liver Transplantation Center, The First Hospital of Shanxi Medical University, Taiyuan, 030001, China
| | - Anhong Zhang
- Department of Hepatobiliary and Pancreatic Surgery, Liver Transplantation Center, The First Hospital of Shanxi Medical University, Taiyuan, 030001, China
| | - Yunfeng Bo
- Department of Pathology, Shanxi Hospital Affiliated to Cancer Hospital, Shanxi Province Cancer Hospital, Chinese Academy of Medical Sciences, Taiyuan, 030001, China
| | - Jun Xu
- Department of Hepatobiliary and Pancreatic Surgery, Liver Transplantation Center, The First Hospital of Shanxi Medical University, Taiyuan, 030001, China.
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Dülger UC, Erdem Ş, Cünük ES, Altıntoprak F. A rare case of small cell neuroendocrine carcinoma of gallbladder origin. J Surg Case Rep 2024; 2024:rjae386. [PMID: 38832057 PMCID: PMC11146206 DOI: 10.1093/jscr/rjae386] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/30/2024] [Accepted: 05/20/2024] [Indexed: 06/05/2024] Open
Abstract
Neuroendocrine carcinomas (NECs) of the gallbladder are very rare and aggressive tumors with poor prognosis. Most of them are poorly differentiated and belong to the small cell type. We report a case of a 59-year-old woman who presented with abdominal pain and distension. Contrast-enhanced computed tomography revealed a large heterogeneous mass in the liver, adjacent to the gallbladder, and omental nodules. CA 19-9 level was elevated and ascitic fluid cytology was suspicious for malignancy. Percutaneous biopsy of the liver mass confirmed the diagnosis of small cell NEC of the gallbladder. The patient was considered inoperable and planned for chemotherapy, but she died 20 days after admission. This case illustrates the diagnostic challenges and the dismal outcome of small cell NEC of the gallbladder. Early detection and multimodal treatment are essential for improving the survival of these patients.
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Affiliation(s)
- Uğur Can Dülger
- Department of General Surgery, Faculty of Medicine, Sakarya University, Sakarya 51400, Sakarya, Turkey
| | - Şahin Erdem
- Department of Pathology, Faculty of Medicine, Sakarya University, Sakarya 51400, Sakarya, Turkey
| | - Emine Sena Cünük
- Department of General Surgery, Faculty of Medicine, Sakarya University, Sakarya 51400, Sakarya, Turkey
| | - Fatih Altıntoprak
- Department of General Surgery, Faculty of Medicine, Sakarya University, Sakarya 51400, Sakarya, Turkey
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Nandy K, Patkar S, Yadav S, Ostwal V, Ramaswamy A, Bhargav P, Goel M. Neuroendocrine neoplasms of the gallbladder: A single institute analysis of outcomes and prognostic factors. J Surg Oncol 2024; 129:1121-1130. [PMID: 38348696 DOI: 10.1002/jso.27600] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/03/2023] [Revised: 01/17/2024] [Accepted: 01/20/2024] [Indexed: 04/24/2024]
Abstract
INTRODUCTION Neuroendocrine neoplasms (NENs) are classified as neuroendocrine tumors (NETs), neuroendocrine carcinomas (NECs), and mixed neuroendocrine and nonneuroendocrine neoplasms (MiNENs) according to World Health Organization classification. We present our experience of NENs of the gallbladder (GB) from a high-volume cancer hospital. MATERIALS AND METHODS The present study is a retrospective analysis of all patients with GB NENs who presented between January 2015 and June 2023. The patient details and treatment received with follow-up were noted. The primary endpoint was overall survival (OS). RESULTS A total of 147 patients were included in the study. The median age was 52 (27-81) years. There was a female predominance (70.7%). NEC was the most common subtype (84.4%) followed by MiNEN (12.9%) and NET (2.7%). The most common stage at presentation was metastatic (70.7%) followed by locally advanced (21.8%), and early disease (7.5%). The median follow-up was 9.92 (1.77-76.06) months. Median OS was 6.14 (3.93-8.35) months. Median OS in patients who received multimodality treatment was 20.20 (17.99-22.41) months versus 4.00 (2.91-5.10) months in those who did not receive it. CONCLUSION GB NENs are rare, but aggressive tumors with NEC being the most common type. Multimodality treatment yields favorable outcomes. However, the development of better systemic therapy is needed to help improve survival further.
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Affiliation(s)
- Kunal Nandy
- Hepatobiliary Division of Department of Surgical Oncology, Tata Memorial Hospital, Homi Bhabha National Institute, Parel, Mumbai, Maharashtra, India
| | - Shraddha Patkar
- Hepatobiliary Division of Department of Surgical Oncology, Tata Memorial Hospital, Homi Bhabha National Institute, Parel, Mumbai, Maharashtra, India
| | - Subhash Yadav
- Department of Pathology, Tata Memorial Hospital, Homi Bhabha National Institute, Parel, Mumbai, Maharashtra, India
| | - Vikas Ostwal
- Department of Medical Oncology, Tata Memorial Hospital, Homi Bhabha National Institute, Parel, Mumbai, Maharashtra, India
| | - Anant Ramaswamy
- Department of Medical Oncology, Tata Memorial Hospital, Homi Bhabha National Institute, Parel, Mumbai, Maharashtra, India
| | - Prabhat Bhargav
- Department of Medical Oncology, Tata Memorial Hospital, Homi Bhabha National Institute, Parel, Mumbai, Maharashtra, India
| | - Mahesh Goel
- Hepatobiliary Division of Department of Surgical Oncology, Tata Memorial Hospital, Homi Bhabha National Institute, Parel, Mumbai, Maharashtra, India
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Zhao ZH, Huang Y, Jiang C, Lv GY, Wang M. Comparative prognosis and risk assessment in gallbladder neuroendocrine neoplasms versus adenocarcinomas. Front Endocrinol (Lausanne) 2024; 15:1326112. [PMID: 38390209 PMCID: PMC10882707 DOI: 10.3389/fendo.2024.1326112] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/22/2023] [Accepted: 01/23/2024] [Indexed: 02/24/2024] Open
Abstract
Background Gallbladder neuroendocrine neoplasms (GB-NENs) are a rare malignant disease, with most cases diagnosed at advanced stages, often resulting in poor prognosis. However, studies regarding the prognosis of this condition and its comparison with gallbladder adenocarcinomas (GB-ADCs) have yet to yield convincing conclusions. Methods We extracted cases of GB-NENs and GB-ADCs from the Surveillance, Epidemiology, and End Results (SEER) database in the United States. Firstly, we corrected differences in clinical characteristics between the two groups using propensity score matching (PSM). Subsequently, we visualized and compared the survival outcomes of the two groups using the Kaplan-Meier method. Next, we employed the least absolute shrinkage and selection operator (LASSO) regression and Cox regression to identify prognostic factors for GB-NENs and constructed two nomograms for predicting prognosis. These nomograms were validated with an internal validation dataset from the SEER database and an external validation dataset from a hospital. Finally, we categorized patients into high-risk and low-risk groups based on their overall survival (OS) scores. Results A total of 7,105 patients were enrolled in the study, comprising 287 GB-NENs patients and, 6,818 GB-ADCs patients. There were substantial differences in clinical characteristics between patients, and GB-NENs exhibited a significantly better prognosis. Even after balancing these differences using PSM, the superior prognosis of GB-NENs remained evident. Independent prognostic factors selected through LASSO and Cox regression were age, histology type, first primary malignancy, tumor size, and surgery. Two nomograms for prognosis were developed based on these factors, and their performance was verified from three perspectives: discrimination, calibration, and clinical applicability using training, internal validation, and external validation datasets, all of which exhibited excellent validation results. Using a cutoff value of 166.5 for the OS nomogram score, patient mortality risk can be identified effectively. Conclusion Patients with GB-NENs have a better overall prognosis compared to those with GB-ADCs. Nomograms for GB-NENs prognosis have been effectively established and validated, making them a valuable tool for assessing the risk of mortality in clinical practice.
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Affiliation(s)
| | | | | | | | - Meng Wang
- Department of Hepatobiliary and Pancreatic Surgery, General Surgery Center, The First Hospital of Jilin University, Changchun, Jilin, China
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Li H, Qiao J, Kou X, Wu C, Liu H, Qiu J. Complete remission of gallbladder neuroendocrine carcinoma with liver metastasis by tislelizumab plus chemotherapy: a case report. Front Oncol 2024; 14:1346290. [PMID: 38357198 PMCID: PMC10864504 DOI: 10.3389/fonc.2024.1346290] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/30/2023] [Accepted: 01/04/2024] [Indexed: 02/16/2024] Open
Abstract
Background Gallbladder neuroendocrine carcinoma (GB-NEC) is an extremely rare cancer with a poor prognosis in the clinic. Although surgical resection remains the primary and preferred therapeutics, many patients are in a late stage and lose the opportunity for surgery. However, due to the extremely low morbidity, the specific treatment guidelines for GB-NEC have not been established. Case presentation A 52-year-old woman was admitted to our hospital with the chief complaint of "almost 1 month after palliative surgery for metastatic gallbladder carcinoma." According to the results of pathological findings and imaging manifestations, the patient was diagnosed with GB-NEC with a clinical stage of pT3N1M1 (IVB). The patient then received tislelizumab plus EP chemotherapy (etoposide 100 mg + cisplatin 30 mg, d1-3) every 3 weeks for 8 cycles from 12 November, 2021, followed by maintenance therapy (tislelizumab alone) every 3 weeks until now. The tumor response was evaluated as complete remission since 13 February, 2023. As of the last follow-up, the patient remains alive, with no complaints of discomfort. Conclusions Gallbladder NEC has no specific symptoms, and the diagnosis is based on pathological and immunohistochemical results. The therapeutic course and efficacy of the case in this study indicates that the application of PD-1 inhibitor might be a feasible therapeutic option for GB-NEC. However, this potential strategy needs validation by further clinical studies in the future.
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Affiliation(s)
- Huafei Li
- School of Life Sciences, Shanghai University, Shanghai, China
| | - Jiaming Qiao
- School of Life Sciences, Shanghai University, Shanghai, China
| | - Xiaoxia Kou
- Department of Oncology Biotherapy, The Third Affiliated Hospital of Navy Medical University, Shanghai, China
| | - Cong Wu
- Clinical Research Unit, The First Affiliated Hospital of Navy Medical University, Shanghai, China
| | - Huiying Liu
- Department of Oncology Biotherapy, The Third Affiliated Hospital of Navy Medical University, Shanghai, China
| | - Jinrong Qiu
- Department of Oncology Biotherapy, The Third Affiliated Hospital of Navy Medical University, Shanghai, China
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Park YM, Seo HI, Noh BG, Kim S, Hong SB, Lee NK, Kim DU, Han SY. Clinicopathological characteristics of extrahepatic biliary neuroendocrine neoplasms in the gallbladder, extrahepatic biliary tract, and ampulla of Vater: A single-center cross-sectional study. Ann Hepatobiliary Pancreat Surg 2023; 27:380-387. [PMID: 37840317 PMCID: PMC10700941 DOI: 10.14701/ahbps.23-045] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/28/2023] [Revised: 06/05/2023] [Accepted: 06/06/2023] [Indexed: 10/17/2023] Open
Abstract
Backgrounds/Aims In 2019, the grading and staging system for neuroendocrine neoplasms (NENs) was significantly changed. In this study, we report the clinicopathological characteristics and surgical outcomes of patients with extrahepatic biliary NENs who underwent curative resection with or without adjuvant treatment. Methods We retrospectively reviewed a database of 16 patients who developed NENs, neuroendocrine carcinoma (NEC), and mixed endocrine non-endocrine neoplasms (MiNENs) after curative resection. Among them, eight patients had ampulla of Vater (AoV) tumors, and eight patients had non-AoV tumors. Results G1 and G2 were more frequently observed in the AoV group than in the non-AoV group (12.5% and 62.5%, respectively). In contrast, NEC and MiNEN were more common in the non-AoV group (50.0%). High Ki-67 index (> 20%) and perineural invasion (PNI) were more frequently observed in the non-AoV group. Advanced age (> 65 years), mitotic count > 20 per 2 mm2, and Ki-67 index > 20% were strongly correlated with patient survival (p = 0.018, 0.009, and 0.044, respectively). Advanced age (> 65 years) and mitotic count > 20 per 2 mm2 were significantly correlated with disease recurrence (p = 0.033 and 0.010, respectively). Conclusions AoV and non-AoV tumors had significant differences in the histologic grade, Ki67, and PNI. Patients with non-AoV tumors had an increased risk for survival and recurrence than those in the AoV group. For extrahepatic biliary NENs, early detection of tumors, adequate surgery, and aggressive adjuvant treatment for high-risk patients are important to achieve long-term survival and prevent disease recurrence.
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Affiliation(s)
- Young Mok Park
- Department of Surgery, Biomedical Research Institute, Pusan National University Hospital, Pusan National University School of Medicine, Busan, Korea
| | - Hyung Il Seo
- Department of Surgery, Biomedical Research Institute, Pusan National University Hospital, Pusan National University School of Medicine, Busan, Korea
| | - Byeong Gwan Noh
- Department of Surgery, Biomedical Research Institute, Pusan National University Hospital, Pusan National University School of Medicine, Busan, Korea
| | - Suk Kim
- Department of Radiology, Biomedical Research Institute, Pusan National University Hospital, Pusan National University School of Medicine, Busan, Korea
| | - Seung Baek Hong
- Department of Radiology, Biomedical Research Institute, Pusan National University Hospital, Pusan National University School of Medicine, Busan, Korea
| | - Nam Kyung Lee
- Department of Radiology, Biomedical Research Institute, Pusan National University Hospital, Pusan National University School of Medicine, Busan, Korea
| | - Dong Uk Kim
- Department of Internal Medicine, Biomedical Research Institute, Pusan National University Hospital, Pusan National University School of Medicine, Busan, Korea
| | - Sung Yong Han
- Department of Internal Medicine, Biomedical Research Institute, Pusan National University Hospital, Pusan National University School of Medicine, Busan, Korea
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Marak JR, Kumar T, Dwivedi S, Khurana R. Neuroendocrine tumor of the gall bladder: A rare case report with review of literature. Radiol Case Rep 2023; 18:3912-3916. [PMID: 37663573 PMCID: PMC10473970 DOI: 10.1016/j.radcr.2023.08.015] [Citation(s) in RCA: 2] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/27/2023] [Revised: 07/10/2023] [Accepted: 08/03/2023] [Indexed: 09/05/2023] Open
Abstract
Neuroendocrine tumor of the gall bladder is an extremely rare malignancy, accounting for only 0.2% of all neuroendocrine tumors. Gall Bladder-Neuroendocrine Tumors (GB-NETs) are mainly diagnosed on histological examination of GB samples after cholecystectomy or after any biliary pathology surgery since it is very difficult to diagnose based on imaging. The overall outcome of gallbladder NET is worse than the adenocarcinoma of the gallbladder. No focused approach towards its treatment is available in literature due to its rarity. We share our experience of gall bladder NET in a 37-year-old female who was successfully managed at our institution.
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Affiliation(s)
- James R. Marak
- Department of Radiodiagnosis, Dr. RMLIMS, Lucknow, Uttar Pradesh, 226010, India
| | - Tushant Kumar
- Department of Radiodiagnosis, Dr. RMLIMS, Lucknow, Uttar Pradesh, 226010, India
| | - Shivam Dwivedi
- Department of Radiodiagnosis, Dr. RMLIMS, Lucknow, Uttar Pradesh, 226010, India
| | - Rohini Khurana
- Department of Radiation Oncology, Dr. RMLIMS, Lucknow, Uttar Pradesh, 226010, India
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Ishikawa M, Ishida K, Machida H, Kato H, Ishizaki S, Nagai T, Matsuyama K, Nozawa Y, Takada-Owada A, Kaneko Y, Sakuma F, Irisawa A. A case of malignant ascites of neuroendocrine carcinoma of the gallbladder diagnosed by endoscopic ultrasound-guided fine-needle aspiration. Diagn Cytopathol 2023; 51:658-661. [PMID: 37501588 DOI: 10.1002/dc.25199] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/02/2023] [Revised: 07/12/2023] [Accepted: 07/18/2023] [Indexed: 07/29/2023]
Affiliation(s)
- Mihoko Ishikawa
- Department of Pathology, Dokkyo Medical University Hospital, Mibu, Japan
| | - Kazuyuki Ishida
- Department of Pathology, Dokkyo Medical University Hospital, Mibu, Japan
- Department of Diagnostic Pathology, Dokkyo Medical University, Mibu, Japan
| | - Hiromi Machida
- Department of Pathology, Dokkyo Medical University Hospital, Mibu, Japan
| | - Hikaru Kato
- Department of Pathology, Dokkyo Medical University Hospital, Mibu, Japan
| | - Satomi Ishizaki
- Department of Pathology, Dokkyo Medical University Hospital, Mibu, Japan
| | - Tamiko Nagai
- Department of Pathology, Dokkyo Medical University Hospital, Mibu, Japan
| | - Kinichi Matsuyama
- Department of Pathology, Dokkyo Medical University Hospital, Mibu, Japan
| | - Yumi Nozawa
- Department of Diagnostic Pathology, Dokkyo Medical University, Mibu, Japan
| | | | - Yuko Kaneko
- Department of Diagnostic Pathology, Dokkyo Medical University, Mibu, Japan
| | - Fumi Sakuma
- Department of Gastroenterology, Dokkyo Medical University, Mibu, Japan
| | - Atsushi Irisawa
- Department of Gastroenterology, Dokkyo Medical University, Mibu, Japan
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12
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Kanda S, Hiyama K, Kirino I, Fukui Y, Terashima H. Effective treatment of gallbladder neuroendocrine carcinoma with nivolumab. Clin Case Rep 2023; 11:e8040. [PMID: 37841882 PMCID: PMC10568049 DOI: 10.1002/ccr3.8040] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/11/2023] [Revised: 09/05/2023] [Accepted: 09/27/2023] [Indexed: 10/17/2023] Open
Abstract
An 89-year-old patient with gallbladder neuroendocrine neoplasms (GB-NENs) and lung cancer metastasis underwent nivolumab monotherapy, resulting in tumor shrinkage. Surgery and adjuvant nivolumab showed efficiency despite low expression of PD-L1.
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Affiliation(s)
| | | | | | - Yasuo Fukui
- Department of SurgeryAtagi HospitalKochiJapan
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13
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Dammak N, Ammar H, Latifa MB, Belkacem O, Mabrouk MB, Ali AB. Gallbladder primary well-differentiated neuroendocrine tumor: A misdiagnosed case. Int J Surg Case Rep 2023; 111:108882. [PMID: 37793232 PMCID: PMC10551650 DOI: 10.1016/j.ijscr.2023.108882] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/29/2023] [Revised: 09/21/2023] [Accepted: 09/23/2023] [Indexed: 10/06/2023] Open
Abstract
INTRODUCTION Gallbladder (GB) Well-Differentiated (WD) Neuroendocrine Tumor (NET) are extremely rare tumors. They represent only about 0.5 % of all Neuroendocrine Neoplasms (NENs). Most GB-NETs are diagnosed after cholecystectomy. They are often misdiagnosed and have good prognosis. CASE PRESENTATION We share our experience of a 42-year-old woman operated on for symptomatic cholelithiasis. We discover on the anatomopathological piece that it was a Grade 1 NET of the GB. Staging scans and octreotide-scans were normal: there is no distant or nodal metastasis. This tumor was classified as T1bN0M0. After multidisciplinary team discussion, additional treatment is deemed unnecessary. She didn't receive any treatment after the surgery. At one year after the cholecystectomy, she is symptom free and there is no sign of recurrence. DISCUSSION The diagnosis of GB-WD NET is difficult at the first evaluation. Surgical management for GB cancer has shown increased survival rates, a similar strategy seems reasonable for GB-NETs. Patients with Grade,1 well-differentiated GB-NETS have an excellent prognosis overall. CONCLUSION Research is required to facilitate earlier diagnosis and to develop management guidelines for GB-NETs.
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Affiliation(s)
- Nouha Dammak
- Department of Digestive and Visceral Surgery, Sahloul University Hospital, Sousse, Tunisia.
| | - Houssem Ammar
- Department of Digestive and Visceral Surgery, Sahloul University Hospital, Sousse, Tunisia
| | - Mahdi Ben Latifa
- Department of Digestive and Visceral Surgery, Sahloul University Hospital, Sousse, Tunisia
| | - Oussama Belkacem
- Pathology Department, Sahloul Hospital, Sousse, Tunisia; Research Laboratory LR21ES03, Medical School of Sousse, University of Sousse, Tunisia
| | - Mohamed Ben Mabrouk
- Department of Digestive and Visceral Surgery, Sahloul University Hospital, Sousse, Tunisia
| | - Ali Ben Ali
- Department of Digestive and Visceral Surgery, Sahloul University Hospital, Sousse, Tunisia
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14
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Iwanaga N, Sugo H, Noro T, Watanobe I, Ogura K. A Case of a Grade 3 Gallbladder Neuroendocrine Tumor With Rapid Recurrence After Curative Resection. Cureus 2023; 15:e47193. [PMID: 38021717 PMCID: PMC10652657 DOI: 10.7759/cureus.47193] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 10/11/2023] [Indexed: 12/01/2023] Open
Abstract
Primary gallbladder neuroendocrine tumor (GB-NET) is extremely rare. Therefore, tumor behavior and adequate treatment in GB-NETs are still unclear. A 74-year-old man without any specific complaints was referred to our hospital cause of gallbladder tumor. Abdominal ultrasonography examination revealed a 22-mm non-pedunculated tumor in the gallbladder body. Contrast-enhanced computed tomography showed a polyp that was enhanced in the arterial phase. The patient underwent gallbladder bed resection and radical lymphadenectomy with a diagnosis of gallbladder carcinoma. Macroscopically, the resected specimen showed a nodular expanding tumor measuring 32×15 mm in the gallbladder body. From the pathological findings, a grade 3 GB-NET was diagnosed. Only cystic lymph node metastasis was observed. The patient was discharged uneventfully, but bone and lymph node metastasis were detected eight months after surgery. We conclude that grade 3 GB-NET shows occasionally malignant biological behavior although NET G3 is distinguished from neuroendocrine carcinoma in the current WHO 2019 classification of NET.
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Affiliation(s)
- Naoki Iwanaga
- Department of General Surgery, Juntendo University Nerima Hospital, Tokyo, JPN
| | - Hiroyuki Sugo
- Department of General Surgery, Juntendo University Nerima Hospital, Tokyo, JPN
| | - Takuji Noro
- Department of General Surgery, Juntendo University Nerima Hospital, Tokyo, JPN
| | - Ikuo Watanobe
- Department of General Surgery, Juntendo University Nerima Hospital, Tokyo, JPN
| | - Kanako Ogura
- Department of Diagnostic Pathology, Juntendo University Nerima Hospital, Tokyo, JPN
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15
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Marak JR, Chauhan A, Anand N, Khurana R. Primary Small Cell Neuroendocrine Carcinoma of nasopharynx with metastatic cervical lymph nodes: an exceedingly rare entity with review of literature. BMJ Case Rep 2023; 16:e255695. [PMID: 37758655 PMCID: PMC10537850 DOI: 10.1136/bcr-2023-255695] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 09/29/2023] Open
Abstract
Small cell neuroendocrine carcinoma of nasopharynx is extremely rare and displays aggressive nature with a poor prognosis. Neuroendocrine tumours rarely arise from the head and neck region and pose a diagnostic and management challenge. In English literature, only 16 cases of primary small cell neuroendocrine carcinoma of nasopharynx have been reported so far; and to the best of our knowledge, this is the seventeenth case and second in the younger age group. Here, we report the case of an adolescent male patient who presented with nasal blockage, repeated episodes of epistaxis and neck swellings. After proper diagnostic workup, the diagnosis of small cell neuroendocrine carcinoma of nasopharynx was made. The patient was treated with chemotherapy, followed by radiotherapy. Imaging investigation executed after the end of the treatment exhibited complete remission of the disease. The patient is kept under active surveillance with no signs of relapse at present.
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Affiliation(s)
- James R Marak
- Radiodiagnosis, Dr Ram Manohar Lohia Institute of Medical Sciences, Lucknow, India
| | - Abhishek Chauhan
- Radiodiagnosis, Dr Ram Manohar Lohia Institute of Medical Sciences, Lucknow, India
| | - Nidhi Anand
- Pathology, Dr Ram Manohar Lohia Institute of Medical Sciences, Lucknow, India
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16
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Vanoli A, Messina A, Gallotti A, Fugazzola P, Adsay V. Well-Differentiated Neuroendocrine Tumor of the Gallbladder with Paraganglioma-Like Features: a Potential Mimicker of Gallbladder Paraganglioma. Endocr Pathol 2023; 34:358-360. [PMID: 37599339 DOI: 10.1007/s12022-023-09784-y] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Accepted: 08/10/2023] [Indexed: 08/22/2023]
Affiliation(s)
- Alessandro Vanoli
- Department of Molecular Medicine, University of Pavia, Pavia, Italy.
| | - Alessia Messina
- Department of Molecular Medicine, University of Pavia, Pavia, Italy
| | - Anna Gallotti
- Institute of Radiology, IRCCS San Matteo Hospital Foundation, Pavia, Italy
| | - Paola Fugazzola
- General and Emergency Surgery, IRCCS San Matteo Hospital Foundation, Pavia, Italy
| | - Volkan Adsay
- Department of Pathology, Koç University School of Medicine and Koç University Research Center for Translational Medicine, Istanbul, Turkey
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17
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Jiang M, Zhang Y. Clinical features and outcomes analysis of Gallbladder neuroendocrine carcinoma. J Cancer Res Ther 2023; 19:910-916. [PMID: 37675716 DOI: 10.4103/jcrt.jcrt_1959_21] [Citation(s) in RCA: 2] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 09/08/2023]
Abstract
Purpose Gallbladder neuroendocrine carcinoma (GB-NEC) is an uncommon and highly malignant tumor. This research aimed to investigate the clinical characteristics and prognostic factors of GB-NEC. Materials and Methods Our survey of case reports from January 2000 to May 2022 screened a total of 84 patients with complete data who received surgical resection for gallbladder NEC. Log-rank generated survival curves using the Kaplan-Meier method for univariate survival analysis. To identify GB-NEC independent prognostic indicators for overall survival (OS), univariate and multivariate Cox proportional hazard analyses were conducted. Results These patients consisted of 25 men and 59 women, with an average age of 60 (range 29-85). Patients under 60 years old made up 44% of the population, while patients over 60 made up 56%. Fifty-three tumors were advanced pathologic TNM stage III and IV. After surgery, 44 patients underwent chemotherapy or radiotherapy. The median OS of 84 patients with GB-NEC was 16.8 months. In univariate and multivariate analysis, tumor size (diameter ≥5 cm), TNM tumor stage, and the receipt of postoperative adjuvant chemotherapy are independent factors influencing the prognosis of patients with GB-NEC. Conclusion Tumor size (diameter ≥5 cm) and TNM tumor stage were independently related to a shorter OS. An enhanced OS was independently linked to receiving postoperative adjuvant chemotherapy.
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Affiliation(s)
- Man Jiang
- Department of Geriatric Gastroenterology, Shandong Provincial Hospital Affiliated to Shandong First Medical University, Jinan, Shandong Province, China
| | - Yijing Zhang
- Department of Geriatric Gastroenterology, Shandong Provincial Hospital Affiliated to Shandong First Medical University, Jinan, Shandong Province, China
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18
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Liao Y, Cao W, Li Z, Xu X, Zhang Y, Liu Z, Miao J, Zhou Y, Zhen Z, Liu D, Li H, Chai L, Wei Y, Zhang X, Zhang L, Wang J, Tian Y. Gallbladder neuroendocrine carcinoma: A report of two cases and literature review. Oncol Lett 2023; 25:229. [PMID: 37153064 PMCID: PMC10157601 DOI: 10.3892/ol.2023.13815] [Citation(s) in RCA: 4] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/26/2022] [Accepted: 02/15/2023] [Indexed: 05/09/2023] Open
Abstract
Gallbladder neuroendocrine carcinoma (GB-NEC) is a rare, aggressive neuroendocrine carcinoma that arises from the gallbladder. Patients with GB-NEC usually have a poor prognosis. The present study described two cases diagnosed with GB-NEC and reviewed the literature to improve knowledge of GB-NEC. The present study reported on two cases of GB-NEC in male patients aged 65 and 66 years, respectively. Both patients underwent surgical resection. Postoperative pathology confirmed that one case had mixed adeno-neuroendocrine carcinoma and the other had large cell neuroendocrine carcinoma. In addition, both patients had uneventful recoveries following surgery and received cisplatin-etoposide combination chemotherapy. The present study summarized the two cases and reviewed the literature to improve understanding of GB-NEC. The results revealed that radiological findings of GB-NEC are non-specific. The present study demonstrated that surgical resection was still the most effective therapy and that postoperative adjuvant chemotherapy could markedly improve the prognosis of patients with GB-NEC.
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Affiliation(s)
- Yong Liao
- Department of Hepatobiliary Surgery, Xingtai People's Hospital of Hebei Medical University, Xingtai, Hebei 054001, P.R. China
| | - Wen Cao
- Department of Neurology, Peking University Third Hospital, Beijing 100191, P.R. China
| | - Zhongkang Li
- Department of Obstetrics and Gynecology, The Second Hospital of Hebei Medical University, Shijiazhuang, Hebei 050000, P.R. China
| | - Xin Xu
- Department of Gynecological Endocrinology, Beijing Obstetrics and Gynecology Hospital, Capital Medical University, Beijing 100191, P.R. China
| | - Ye Zhang
- Department of Obstetrics and Gynecology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, Henan 450052, P.R. China
| | - Zhihu Liu
- Department of Hepatobiliary Surgery, Xingtai People's Hospital of Hebei Medical University, Xingtai, Hebei 054001, P.R. China
| | - Jie Miao
- Department of Pathology, Xingtai People's Hospital of Hebei Medical University, Xingtai, Hebei 054001, P.R. China
| | - Yang Zhou
- Department of Pathology, Xingtai People's Hospital of Hebei Medical University, Xingtai, Hebei 054001, P.R. China
| | - Zhongguang Zhen
- Department of Hepatobiliary Surgery, Xingtai People's Hospital of Hebei Medical University, Xingtai, Hebei 054001, P.R. China
| | - Dengxiang Liu
- Department of Hepatobiliary Surgery, Xingtai People's Hospital of Hebei Medical University, Xingtai, Hebei 054001, P.R. China
| | - Hui Li
- Department of Hepatobiliary Surgery, Xingtai People's Hospital of Hebei Medical University, Xingtai, Hebei 054001, P.R. China
| | - Lichao Chai
- Department of Nuclear Medicine, Xingtai People's Hospital of Hebei Medical University, Xingtai, Hebei 054001, P.R. China
| | - Yan Wei
- Department of Nuclear Medicine, Xingtai People's Hospital of Hebei Medical University, Xingtai, Hebei 054001, P.R. China
| | - Xiaochong Zhang
- Department of Hepatobiliary Surgery, Xingtai People's Hospital of Hebei Medical University, Xingtai, Hebei 054001, P.R. China
| | - Le Zhang
- Department of Oral Surgery, Hebei Provincial Eye Hospital, Xingtai, Hebei 054001, P.R. China
| | - Jitao Wang
- Department of Hepatobiliary Surgery, Xingtai People's Hospital of Hebei Medical University, Xingtai, Hebei 054001, P.R. China
- Dr Jitao Wang, Department of Hepatobiliary Surgery, Xingtai People's Hospital of Hebei Medical University, Hongxing Street, Xingtai, Hebei 054001, P.R. China, E-mail:
| | - Yanpeng Tian
- Department of Obstetrics and Gynecology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, Henan 450052, P.R. China
- Correspondence to: Dr Yanpeng Tian, Department of Obstetrics and Gynecology, The First Affiliated Hospital of Zhengzhou University, 1 Jianshe East Road, Zhengzhou, Henan 450052, P.R. China, E-mail:
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19
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Das S, Prakash S, Shaikh O, Reddy A, Kumbhar U. Small Cell Carcinoma of the Gallbladder. Cureus 2023; 15:e38444. [PMID: 37273321 PMCID: PMC10234611 DOI: 10.7759/cureus.38444] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 05/02/2023] [Indexed: 06/06/2023] Open
Abstract
Primary neuroendocrine carcinoma of the gallbladder (GB) is a rare, highly dismal lethal disease with a fatal prognosis. A 45-year-old female presented with right upper abdomen pain and multiple vomiting episodes. Imaging studies showed diffuse thickening of the wall of the GB with locoregional invasion into the nearby structures with extensive abdominal lymph node metastasis and arteriovenous encasements. Ultrasound-guided fine-needle aspiration was done, which was diagnostic of small cell carcinoma of the GB. The patient was planned for palliative chemotherapy. A small cell variant of neuroendocrine carcinoma of the GB is a rare entity with a moribund lethality associated with it. Patients are diagnosed in advanced stages with not many treatment modalities to offer. Usually, patients are treated with palliative chemotherapy.
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Affiliation(s)
- Snehasis Das
- Surgery, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry, IND
| | - Sagar Prakash
- Surgery, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry, IND
| | - Oseen Shaikh
- Surgery, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry, IND
| | - Abhinaya Reddy
- Surgery, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry, IND
| | - Uday Kumbhar
- Surgery, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry, IND
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20
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Fick A, Tran K, Kandpal H, Burge M, Chandrasegaram M. An Unusual Case of Large Cell Neuroendocrine Cancer of the Gallbladder With Mixed Adenocarcinoma Component in a Patient With Pancreatobiliary Maljunction. Cureus 2023; 15:e37398. [PMID: 37056222 PMCID: PMC10089643 DOI: 10.7759/cureus.37398] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 03/29/2023] [Indexed: 04/15/2023] Open
Abstract
Gallbladder neuroendocrine tumors (GB-NETs) and gallbladder neuroendocrine carcinomas (GB-NECs) are rare forms of neuroendocrine neoplasms (NENs). Most GB-NENs present as incidental findings or as gallbladder polyps in the course of investigation of nonspecific symptoms such as upper abdominal pain and nausea. Given the rarity of GB-NENs, only a few small case series are currently available describing this entity, and even fewer guiding consensus on standard treatment and the role of adjuvant therapy. We present the case of a 65-year-old South American female referred for a chronic history of intermittent post-prandial epigastric pain, bloating, nausea, and occasional diarrhea. Pancreaticobiliary maljunction with dilation was present and she was diagnosed with primary gallbladder large cell neuroendocrine carcinoma (GB-LCNEC) mixed with a minor component of gallbladder adenocarcinoma.
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Affiliation(s)
- Anton Fick
- General Surgery, The Prince Charles Hospital, Brisbane, AUS
| | - Kayla Tran
- Pathology, The Prince Charles Hospital, Brisbane, AUS
| | - Harsh Kandpal
- Radiology, The Prince Charles Hospital, Brisbane, AUS
| | - Matthew Burge
- Medical Oncology, The Prince Charles Hospital, Brisbane, AUS
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21
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Pandey L, Elhence A, Gupta S, Joseph D, Pasricha R, Gupta M. Small-cell carcinoma of the gall bladder: Report of three cases and review of the literature. Med J Armed Forces India 2022; 78:S330-S334. [PMID: 36147409 PMCID: PMC9485769 DOI: 10.1016/j.mjafi.2020.04.003] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/12/2019] [Accepted: 04/03/2020] [Indexed: 11/23/2022] Open
Abstract
Gallbladder (GB) Small Cell Carcinoma (SCC) is an uncommon entity with very poor prognosis. There is a paucity of literature regarding its natural history and management, with only 73 prior cases reported in the world literature. In this case report, we present three cases of SCC of the GB with varied presentations, clinical course, management, and outcomes along with a brief review of the available literature on this subject.
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Affiliation(s)
- Laxman Pandey
- Senior Resident (Radiation Oncology), All India Institute of Medical Sciences, Rishikesh, India
| | - Aditya Elhence
- Senior Resident (Radiation Oncology), All India Institute of Medical Sciences, Rishikesh, India
| | - Sweety Gupta
- Assistant Professor (Radiation Oncology), All India Institute of Medical Sciences, Rishikesh, India
| | - Deepa Joseph
- Associate Professor (Radiation Oncology), All India Institute of Medical Sciences, Rishikesh, India
| | - Rajesh Pasricha
- Additional Professor (Radiation Oncology), All India Institute of Medical Sciences, Rishikesh, India
| | - Manoj Gupta
- Professor (Radiation Oncology), All India Institute of Medical Sciences, Rishikesh, India
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22
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Rennie AT, Halbreich SL. Rare Case of Gallbladder Neuroendocrine Carcinoma. Cureus 2022; 14:e28531. [PMID: 36185880 PMCID: PMC9517954 DOI: 10.7759/cureus.28531] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/10/2022] [Accepted: 08/27/2022] [Indexed: 11/11/2022] Open
Abstract
Neuroendocrine tumors (NET) are very uncommon, though when seen, they are typically found within the gastrointestinal tract. Rarely NETs can be seen within the lumen of the gallbladder. An 83-year-old woman presented to our hospital with vague abdominal pain and distention. She was found to have a mass within her gallbladder on imaging, and she was treated with surgical resection. Pathology revealed a gallbladder NET with lymphovascular involvement. NETs of the gallbladder remain a rare occurrence with a limited number of previous case reports.
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23
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Noh BG, Seo HI, Park YM, Kim S, Hong SB, Lee SJ. Complete resection of large-cell neuroendocrine and hepatocellular carcinoma of the liver: A case report. World J Clin Cases 2022; 10:8277-8283. [PMID: 36159537 PMCID: PMC9403669 DOI: 10.12998/wjcc.v10.i23.8277] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/16/2022] [Revised: 06/07/2022] [Accepted: 07/11/2022] [Indexed: 02/06/2023] Open
Abstract
BACKGROUND Combined tumors comprising large-cell neuroendocrine carcinoma and hepatocellular carcinoma have been rarely reported in the literature.
CASE SUMMARY We report a case of a 73-year-old woman with chronic hepatitis B suspected to have a malignant hepatic mass (segment 3; size, 4.5 cm) and lymph node metastasis based on computed tomography and magnetic resonance imaging. Despite being Child-Pugh class A, esophageal varices were present. She underwent left lateral sectionectomy and lymph node dissection. Pathological examination revealed a collision tumor consisting of large-cell neuroendocrine (90%) and hepatocellular (10%) carcinomas. The combined carcinoma had metastasized to one of the three lymph nodes excised. The patient recovered without any postoperative complications and was discharged in good condition on postoperative day 13. Adjuvant chemotherapy was not performed. No recurrence occurred during a follow-up period of 24 mo.
CONCLUSION To improve the therapeutic management of combined tumors in the liver, it is necessary to discuss each clinical experience and consider an appropriate method for the preoperative diagnosis and treatment.
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Affiliation(s)
- Byeong Gwan Noh
- Division of HBP Surgery and Transplantation, Department of Surgery, Pusan National University School of Medicine, Biomedical Research Institute, Pusan National University Hospital, Busan 49241, South Korea
| | - Hyung-Il Seo
- Division of HBP Surgery and Transplantation, Department of Surgery, Pusan National University School of Medicine, Biomedical Research Institute, Pusan National University Hospital, Busan 49241, South Korea
| | - Young Mok Park
- Division of HBP Surgery and Transplantation, Department of Surgery, Pusan National University School of Medicine, Biomedical Research Institute, Pusan National University Hospital, Busan 49241, South Korea
| | - Suk Kim
- Department of Radiology, Pusan National University School of Medicine, Biomedical Research Institute, Pusan National University Hospital, Busan 49241, South Korea
| | - Seung Baek Hong
- Department of Radiology, Pusan National University School of Medicine, Biomedical Research Institute, Pusan National University Hospital, Busan 49241, South Korea
| | - So Jeong Lee
- Department of Pathology, Pusan National University School of Medicine, Biomedical Research Institute, Pusan National University Hospital, Busan 49241, South Korea
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24
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Cai XC, Wu SD. Gallbladder neuroendocrine carcinoma diagnosis, treatment and prognosis based on the SEER database: A literature review. World J Clin Cases 2022; 10:8212-8223. [PMID: 36159526 PMCID: PMC9403678 DOI: 10.12998/wjcc.v10.i23.8212] [Citation(s) in RCA: 10] [Impact Index Per Article: 3.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/18/2022] [Revised: 04/21/2022] [Accepted: 07/11/2022] [Indexed: 02/06/2023] Open
Abstract
BACKGROUND Gallbladder neuroendocrine carcinoma (GB-NEC) has a low incidence rate; therefore, its clinical characteristics, diagnosis, treatment and prognosis are not well explored.
AIM To review recent research and analyze corresponding data in the Surveillance Epidemiology and End Results (SEER) database.
METHODS Data of GB-NEC (n = 287) and gallbladder adenocarcinoma (GB-ADC) (n = 19 484) patients from 1975 to 2016 were extracted from the SEER database. Survival analysis was performed using Kaplan–Meier and Cox proportional hazards regression. P < 0.05 was considered statistically significant. We also reviewed 108 studies retrieved from PubMed and Reference Citation Analysis (https://www.referencecitationanalysis.com/). The keywords used for the search were: "(Carcinoma, Neuroendocrine) AND (Gallbladder Neoplasms)".
RESULTS The GB-NEC incidence rate was 1.6% (of all gallbladder carcinomas), male to female ratio was 1:2 and the median survival time was 7 mo. The 1-, 2-, 3- and 5-year overall survival (OS) was 36.6%, 17.8%, 13.2% and 7.3% respectively. Serum chromogranin A levels may be a specific tumor marker for the diagnosis of GB-NEC. Elevated carcinoembryonic antigen, carbohydrate antigen (CA)-19-9 and CA-125 levels were associated with poor prognosis. Age [hazard ratio (HR) = 1.027, 95% confidence interval (CI): 1.006–1.047, P = 0.01] and liver metastasis (HR = 3.055, 95% CI: 1.839–5.075, P < 0.001) are independent prognostic risk factors for OS. Patients with advanced GB-NEC treated with surgical resection combined with radiotherapy and/or chemotherapy may have a better prognosis than those treated with surgical resection alone. There was no significant difference in OS between GB-NEC and GB-ADC.
CONCLUSION The clinical manifestations and prognosis of GB-NEC are similar to GB-ADC, but the treatment is completely different. Early diagnosis and treatment are the top priorities.
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Affiliation(s)
- Xing-Chen Cai
- Department of Hepato-Pancreato-Billiary Surgery, The affiliated Lihuili Hospital, Ningbo University, Ningbo, Zhejiang, China
| | - Sheng-Dong Wu
- Department of Hepato-Pancreato-Billiary Surgery, The affiliated Lihuili Hospital, Ningbo University, Ningbo, Zhejiang, China
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25
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Vidal Panduro DA, Zegarra Buitron E, Cochella Tizon OJ, Morales Luna DA. Neuroendocrine Carcinoma of the Gallbladder. Cureus 2022; 14:e27022. [PMID: 35989827 PMCID: PMC9387875 DOI: 10.7759/cureus.27022] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 06/24/2022] [Indexed: 11/10/2022] Open
Abstract
Neuroendocrine carcinoma of the gallbladder (NECGB) is a rare, aggressive tumor with a poor prognosis. There are two main categories, well-differentiated NECGB and poorly differentiated NECGB, the latter with a worse prognosis. The clinical presentation is non-specific, but the occurrence is more frequent in women with cholelithiasis. Histologic and immuno-histochemical confirmation is required to establish the diagnosis. Treatment is primarily surgery with or without adjuvant chemotherapy. We present the case of a 43-year-old woman with pain in the right upper quadrant, diagnosed with NECGB following cholecystectomy. Subsequently, she received cycles of chemotherapy.
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26
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Wang Y, Huang B, Fu Q, Wang J, Ye M, Hu M, Qu K, Liu K, Hu X, Wei S, Sun K, Xiao W, Zhang B, Li H, Li J, Zhang Q, Liang T. Comprehensive Clinical Analysis of Gallbladder Neuroendocrine Neoplasms: A Large-Volume Multicenter Study During One Decade. Ann Surg Oncol 2022; 29:7619-7630. [PMID: 35849293 DOI: 10.1245/s10434-022-12107-w] [Citation(s) in RCA: 7] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/02/2022] [Accepted: 06/05/2022] [Indexed: 02/06/2023]
Abstract
BACKGROUND This study aimed to comprehensively investigate the clinicopathologic characteristics and therapeutic situations of gallbladder neuroendocrine neoplasms (GB-NENs) in the real world via a multicenter, large-scale cohort study. METHODS The study searched for patients in 143 hospitals in China and enrolled 154 patients with GB-NENs diagnosed in 40 hospitals between 2004 and 2021. Clinicopathologic characteristics and therapeutic approaches were analyzed retrospectively. RESULTS The median age at the initial diagnosis of the patients with GB-NENs was 63 years (range 33-83 years), and 61.7% of the patients were women. Tumor-node-metastasis staging classified 92 patients as stage 3 or above. Based on the 2019 World Health Organization classification, 96 cases (62.3%) were confirmed pathologically as poorly differentiated neuroendocrine carcinomas, 13 cases (8.4%) as well-differentiated neuroendocrine tumors, and 45 cases as mixed neuroendocrine-non-neuroendocrine neoplasms. The liver was the most frequent metastatic site. Immunohistochemistry showed that synaptophysin was most frequently positive (80.4%), followed by chromogranin A (61.7%), and CD56 (58.4%). Computed tomography and magnetic resonance imaging showed more common clear boundaries (25/39 cases) and invasive growth features (27 cases). None of these cases had an accurate diagnosis before surgery, with a misdiagnosis rate of 100%. Surgical resection is the main treatment, and platinum-based chemotherapeutic regimens were preferred as adjuvant therapies for patients with GB-NENs. The available survival data for 74 patients showed an overall survival rate of 59% at 1 year, 33% at 3 years, and 29% at 5 years. No significant difference was found between the patients treated with and those treated without adjuvant chemotherapy. CONCLUSIONS Gallbladder neuroendocrine neoplasms have high malignancy and a poor prognosis. Importantly, this large-scale cohort study significantly improves our understanding of GB-NENs and will benefit the exploration of its mechanism and treatment modes. Further investigation is necessary to explore the management of this disease.
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Affiliation(s)
- Yangyang Wang
- Department of Hepatobiliary and Pancreatic Surgery, The First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, China.,Zhejiang Provincial Key Laboratory of Pancreatic Disease, The First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, China
| | - Bingfeng Huang
- Department of Hepatobiliary and Pancreatic Surgery, The First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, China
| | - Qihan Fu
- Zhejiang Clinical Research Center of Hepatobiliary and Pancreatic Diseases, Hangzhou, China.,Zhejiang University Cancer Center, Hangzhou, China.,Department of Medical Oncology, The First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, China
| | - Jianing Wang
- Department of Hepatobiliary and Pancreatic Surgery, The First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, China.,Zhejiang Provincial Key Laboratory of Pancreatic Disease, The First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, China
| | - Mao Ye
- Department of Hepatobiliary and Pancreatic Surgery, The First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, China.,Zhejiang Provincial Key Laboratory of Pancreatic Disease, The First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, China
| | - Manyi Hu
- Department of Hepatobiliary and Pancreatic Surgery, The First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, China
| | - Kai Qu
- Department of Hepatobiliary Surgery, The First Affiliated Hospital of Xi'an Jiaotong University, Xi'an, China
| | - Kai Liu
- Department of Hepatobiliary and Pancreatic Surgery, The First Hospital of Jilin University, Changchun, China
| | - Xiao Hu
- Department of Hepatobiliary Pancreatic Surgery, The Affiliated Hospital of Qingdao University, Qingdao, China
| | - Shumei Wei
- Department of Pathology, The Second Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, China
| | - Ke Sun
- Department of Pathology, The First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, China
| | - Wenbo Xiao
- Department of Radiology, The First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, China
| | - Bo Zhang
- Department of General Surgery, Shenzhen University Luohu People's Hospital, Shenzhen, China
| | - Haijun Li
- Department of General Surgery, Shenzhen University Luohu People's Hospital, Shenzhen, China
| | - Jingsong Li
- The Engineering Research Center of EMR and Intelligent Expert System, Ministry of Education, College of Biomedical Engineering and Instrument Science, Zhejiang University, Hangzhou, China.,Research Center for Healthcare Data Science, Zhejiang Lab, Hangzhou, China
| | - Qi Zhang
- Department of Hepatobiliary and Pancreatic Surgery, The First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, China. .,Zhejiang Provincial Key Laboratory of Pancreatic Disease, The First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, China. .,Zhejiang Clinical Research Center of Hepatobiliary and Pancreatic Diseases, Hangzhou, China. .,Zhejiang University Cancer Center, Hangzhou, China.
| | - Tingbo Liang
- Department of Hepatobiliary and Pancreatic Surgery, The First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, China. .,Zhejiang Provincial Key Laboratory of Pancreatic Disease, The First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, China. .,Zhejiang Clinical Research Center of Hepatobiliary and Pancreatic Diseases, Hangzhou, China. .,Zhejiang University Cancer Center, Hangzhou, China.
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27
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Nuthulaganti SR, Sharma R, Candula N, Gujarathi R, Saremian J. A Rare Case of Stage IV Mixed Neuroendocrine Small Cell and Adenosquamous Cell Carcinoma of the Gallbladder. Cureus 2022; 14:e26858. [PMID: 35974840 PMCID: PMC9375118 DOI: 10.7759/cureus.26858] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/23/2022] [Accepted: 07/14/2022] [Indexed: 11/09/2022] Open
Abstract
Gallbladder (GB) carcinoma is the fifth most common type of gastrointestinal cancer. Although a majority of these cancers are found to be adenocarcinomas, we present a rare case in which the GB carcinoma was found to have mixed histology with both small cell neuroendocrine carcinoma and adenosquamous cell carcinoma.
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28
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Zheng BH, Zhang C, Wan WZ, Sun WT, Cheng X, Ni XJ, Ni XL, Suo T, Liu H, Shen S, Liu HB. The clinical and prognostic factors for biliary neuroendocrine neoplasm: a study based on the SEER database. BMC Surg 2022; 22:253. [PMID: 35768809 PMCID: PMC9245279 DOI: 10.1186/s12893-022-01689-7] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/20/2021] [Accepted: 06/06/2022] [Indexed: 12/15/2022] Open
Abstract
BACKGROUND In this study, we aimed at elucidating the postoperative survival and prognostic factors in patients with biliary neuroendocrine neoplasm (NEN). METHODS Cases of biliary system NEN and adenocarcinoma from 1975 to 2016 were extracted from the Surveillance, Epidemiology, and End Results (SEER) database. A propensity score matching (PSM) method was used to adjust baseline differences in clinicopathological characteristics in our analysis. The Kaplan-Meier analysis was carried out for survival analysis. RESULTS A total of 233 patients with biliary system NEN were enrolled in this study, of which 119 patients' lesions located in gallbladder, while the others' located in bile duct. The postoperative overall survival of bile duct NEN is significantly longer than that of gallbladder NEN (P < 0.001). For gallbladder NENs, surgery method (P = 0.020) and lymph node metastasis (P = 0.018) were identified as independent prognostic factors. In terms of ampulla of vater (AOV) NENs, age (P = 0.017) and lymph node metastasis (P = 0.006) were identified as independent prognostic factors, while grade (P = 0.002) and lymph node metastasis (P = 0.036) were identified as independent prognostic factors for extrahepatic bile duct (EBD) NENs. PSM analysis indicated that patients with biliary duct NENs have a better postoperative prognosis than biliary duct adenocarcinoma. CONCLUSIONS Patients with NEN have better overall survival than patients with adenocarcinoma. Gallbladder NEN has an adverse prognosis than that of biliary tract NEN. The pathological subtype, differentiation, lymph node metastasis, surgery method, and lymph node resection could affect the postoperative prognosis of the gallbladder and biliary tract NEN.
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Affiliation(s)
- Bo-Hao Zheng
- Department of General Surgery, Zhongshan Hospital, Fudan University, 180 Fenglin Road, Shanghai, 200032 China
- Biliary Tract Disease Center, Zhongshan Hospital, Fudan University, 180 Fenglin Road, Shanghai, 200032 China
- Biliary Tract Disease Institute, Fudan University, 180 Fenglin Road, Shanghai, 200032 China
- Cancer Center, Zhongshan Hospital, 180 Fenglin Road, 200032 Shanghai, China
- Shanghai Engineering Research Center of Biliary Tract Minimal Invasive Surgery and Materials, Shanghai, China
| | - Cheng Zhang
- Department of General Surgery, Zhongshan Hospital, Fudan University, 180 Fenglin Road, Shanghai, 200032 China
- Biliary Tract Disease Center, Zhongshan Hospital, Fudan University, 180 Fenglin Road, Shanghai, 200032 China
- Biliary Tract Disease Institute, Fudan University, 180 Fenglin Road, Shanghai, 200032 China
- Cancer Center, Zhongshan Hospital, 180 Fenglin Road, 200032 Shanghai, China
- Shanghai Engineering Research Center of Biliary Tract Minimal Invasive Surgery and Materials, Shanghai, China
| | - Wen-Ze Wan
- Department of General Surgery, Zhongshan Hospital, Fudan University, 180 Fenglin Road, Shanghai, 200032 China
- Biliary Tract Disease Center, Zhongshan Hospital, Fudan University, 180 Fenglin Road, Shanghai, 200032 China
- Biliary Tract Disease Institute, Fudan University, 180 Fenglin Road, Shanghai, 200032 China
- Cancer Center, Zhongshan Hospital, 180 Fenglin Road, 200032 Shanghai, China
- Shanghai Engineering Research Center of Biliary Tract Minimal Invasive Surgery and Materials, Shanghai, China
| | - Wen-Tao Sun
- Department of General Surgery, Zhongshan Hospital, Fudan University, 180 Fenglin Road, Shanghai, 200032 China
- Biliary Tract Disease Center, Zhongshan Hospital, Fudan University, 180 Fenglin Road, Shanghai, 200032 China
- Biliary Tract Disease Institute, Fudan University, 180 Fenglin Road, Shanghai, 200032 China
- Cancer Center, Zhongshan Hospital, 180 Fenglin Road, 200032 Shanghai, China
- Shanghai Engineering Research Center of Biliary Tract Minimal Invasive Surgery and Materials, Shanghai, China
| | - Xi Cheng
- Department of General Surgery, Zhongshan Hospital, Fudan University, 180 Fenglin Road, Shanghai, 200032 China
- Biliary Tract Disease Center, Zhongshan Hospital, Fudan University, 180 Fenglin Road, Shanghai, 200032 China
- Biliary Tract Disease Institute, Fudan University, 180 Fenglin Road, Shanghai, 200032 China
- Cancer Center, Zhongshan Hospital, 180 Fenglin Road, 200032 Shanghai, China
- Shanghai Engineering Research Center of Biliary Tract Minimal Invasive Surgery and Materials, Shanghai, China
| | - Xiao-Jian Ni
- Department of General Surgery, Zhongshan Hospital, Fudan University, 180 Fenglin Road, Shanghai, 200032 China
- Biliary Tract Disease Center, Zhongshan Hospital, Fudan University, 180 Fenglin Road, Shanghai, 200032 China
- Biliary Tract Disease Institute, Fudan University, 180 Fenglin Road, Shanghai, 200032 China
- Cancer Center, Zhongshan Hospital, 180 Fenglin Road, 200032 Shanghai, China
- Shanghai Engineering Research Center of Biliary Tract Minimal Invasive Surgery and Materials, Shanghai, China
| | - Xiao-Ling Ni
- Department of General Surgery, Zhongshan Hospital, Fudan University, 180 Fenglin Road, Shanghai, 200032 China
- Biliary Tract Disease Center, Zhongshan Hospital, Fudan University, 180 Fenglin Road, Shanghai, 200032 China
- Biliary Tract Disease Institute, Fudan University, 180 Fenglin Road, Shanghai, 200032 China
- Cancer Center, Zhongshan Hospital, 180 Fenglin Road, 200032 Shanghai, China
- Shanghai Engineering Research Center of Biliary Tract Minimal Invasive Surgery and Materials, Shanghai, China
| | - Tao Suo
- Department of General Surgery, Zhongshan Hospital, Fudan University, 180 Fenglin Road, Shanghai, 200032 China
- Biliary Tract Disease Center, Zhongshan Hospital, Fudan University, 180 Fenglin Road, Shanghai, 200032 China
- Biliary Tract Disease Institute, Fudan University, 180 Fenglin Road, Shanghai, 200032 China
- Cancer Center, Zhongshan Hospital, 180 Fenglin Road, 200032 Shanghai, China
- Shanghai Engineering Research Center of Biliary Tract Minimal Invasive Surgery and Materials, Shanghai, China
| | - Han Liu
- Department of General Surgery, Zhongshan Hospital, Fudan University, 180 Fenglin Road, Shanghai, 200032 China
- Biliary Tract Disease Center, Zhongshan Hospital, Fudan University, 180 Fenglin Road, Shanghai, 200032 China
- Biliary Tract Disease Institute, Fudan University, 180 Fenglin Road, Shanghai, 200032 China
- Cancer Center, Zhongshan Hospital, 180 Fenglin Road, 200032 Shanghai, China
- Shanghai Engineering Research Center of Biliary Tract Minimal Invasive Surgery and Materials, Shanghai, China
| | - Sheng Shen
- Department of General Surgery, Zhongshan Hospital, Fudan University, 180 Fenglin Road, Shanghai, 200032 China
- Biliary Tract Disease Center, Zhongshan Hospital, Fudan University, 180 Fenglin Road, Shanghai, 200032 China
- Biliary Tract Disease Institute, Fudan University, 180 Fenglin Road, Shanghai, 200032 China
- Cancer Center, Zhongshan Hospital, 180 Fenglin Road, 200032 Shanghai, China
- Shanghai Engineering Research Center of Biliary Tract Minimal Invasive Surgery and Materials, Shanghai, China
| | - Hou-Bao Liu
- Department of General Surgery, Zhongshan Hospital, Fudan University, 180 Fenglin Road, Shanghai, 200032 China
- Biliary Tract Disease Center, Zhongshan Hospital, Fudan University, 180 Fenglin Road, Shanghai, 200032 China
- Biliary Tract Disease Institute, Fudan University, 180 Fenglin Road, Shanghai, 200032 China
- Cancer Center, Zhongshan Hospital, 180 Fenglin Road, 200032 Shanghai, China
- Shanghai Engineering Research Center of Biliary Tract Minimal Invasive Surgery and Materials, Shanghai, China
- Department of General Surgery, Biliary Tract Disease Institute, Zhongshan Hospital, Fudan University, 180 Fenglin Road, Shanghai, 200032 China
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Takeda Y, Kobayashi N, Kessoku T, Okubo N, Suzuki A, Tokuhisa M, Miwa H, Udaka N, Ichikawa Y. Case reports: chemoradiotherapy for locally advanced neuroendocrine carcinoma of the gallbladder. Clin J Gastroenterol 2022; 15:803-808. [PMID: 35653037 PMCID: PMC9334423 DOI: 10.1007/s12328-022-01645-1] [Citation(s) in RCA: 3] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/28/2022] [Accepted: 05/09/2022] [Indexed: 11/11/2022]
Abstract
Neuroendocrine carcinoma (NEC) is a rare subtype of malignant gallbladder tumor. Although surgical resection is the only potentially curative therapy for gallbladder NEC, most cases are surgically unresectable because of advanced stage disease and/or biologically aggressive behavior. The standard palliative treatment for malignant gallbladder tumors is chemotherapy; however, the efficacy of chemoradiotherapy in the treatment of gallbladder tumors is controversial. Here, we report a case of gallbladder NEC that showed a durable response to chemoradiotherapy. A 68-year-old Japanese man presented with a huge gallbladder tumor with liver and duodenal invasion. Pathological findings revealed poorly differentiated NEC of the gallbladder. After seven cycles of chemotherapy comprising cisplatin and irinotecan, computed tomography (CT) revealed remarkable tumor shrinkage, but an enlarged portal lymph node. The patient was treated with 50.4 Gy in 28 fractions with two cycles of cisplatin and etoposide. After chemoradiotherapy, the enlarged lymph node also decreased in size. Maximum standardized uptake value of fluorodeoxyglucose-positron emission tomography/CT(FDG-PET/CT) changed from 8.2 to physiological accumulation. We defined this condition as a complete response on both enhanced CT and FDG-PET/CT; therefore, we did not perform systemic treatment and only observed his condition. This patient remained healthy with no recurrence at 3 years after chemoradiotherapy.
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Affiliation(s)
- Yuma Takeda
- Department of Oncology, Yokohama City University School of Medicine Graduate School of Medicine, Yokohama, Japan
| | - Noritoshi Kobayashi
- Department of Oncology, Yokohama City University School of Medicine Graduate School of Medicine, Yokohama, Japan.
| | - Takaomi Kessoku
- Department of Palliative Medicine, Yokohama City University Hospital, Yokohama, Japan.,Department of Gastroenterology and Hepatology, Yokohama City University School of Medicine Graduate School of Medicine, Yokohama, Japan
| | - Naoki Okubo
- Department of Oncology, Yokohama City University School of Medicine Graduate School of Medicine, Yokohama, Japan
| | - Akihiro Suzuki
- Department of Oncology, Yokohama City University School of Medicine Graduate School of Medicine, Yokohama, Japan
| | - Motohiko Tokuhisa
- Department of Oncology, Yokohama City University School of Medicine Graduate School of Medicine, Yokohama, Japan
| | - Haruo Miwa
- Gastroenterological Center, Yokohama City University Medical Center, Yokohama, Japan
| | - Naoko Udaka
- Department of Pathology, Yokohama City University Hospital, 3-9, Fuku-ura, Kanazawa-ku, Yokohama, 2360004, Japan
| | - Yasushi Ichikawa
- Department of Oncology, Yokohama City University School of Medicine Graduate School of Medicine, Yokohama, Japan
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30
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Kumar A, Kumar B, Muthu GS, Mitra S. 68Ga-DOTANOC PET/CT Detects a Rare Case of Metastatic Neuroendocrine Neoplasm of the Gallbladder. Clin Nucl Med 2022; 47:539-540. [PMID: 35384899 DOI: 10.1097/rlu.0000000000004142] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/26/2022]
Abstract
ABSTRACT Gallbladder neuroendocrine neoplasms (NENs) are rare tumors of the biliary system. These neoplasms express somatostatin receptors, and hence radiolabeled somatostatin analog 68Ga-DOTANOC is used as a PET radiotracer in detection and staging. Gallbladder NEN cannot be differentiated from an adenocarcinoma of the gallbladder based on clinical symptoms or routine radiological imaging such as ultrasound or CT. These are either diagnosed postcholecystectomy or after biopsy from primary or metastatic sites. We present a rare case of gallbladder NEN detected on 68Ga-DOTANOC PET/CT.
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31
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A Rare Case of Pure Primary Large Cell Neuroendocrine Carcinoma of the Gallbladder. Case Rep Oncol Med 2022; 2022:6956046. [PMID: 35637677 PMCID: PMC9148250 DOI: 10.1155/2022/6956046] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/01/2021] [Revised: 04/12/2022] [Accepted: 04/29/2022] [Indexed: 11/17/2022] Open
Abstract
Primary large cell neuroendocrine carcinoma (LCNEC) of the gallbladder is a rare malignancy which is often associated with non-LCNEC histologic components. Histologically “pure” LCNECs of the gallbladder are exceedingly rare with only 15 cases reported in the medical literature. Clinically, LCNECs present with abdominal pain and jaundice and follow an aggressive course with patients surviving a median of 15 months following initial diagnosis. To our knowledge, we present the 16th case of a histologically pure LCNEC in a 62-year-old African American male who was successfully treated surgically. After discharge, he was subsequently lost to follow-up. Due to the extreme rarity of such disease entity, accurate diagnosis and proper management are essential for the best clinical outcome.
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32
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Hsiao TH, Wu CC, Tseng HH, Chen JH. Synchronous but separate neuroendocrine tumor and high-grade dysplasia/adenoma of the gall bladder: A case report. World J Clin Cases 2022; 10:2322-2329. [PMID: 35321155 PMCID: PMC8895191 DOI: 10.12998/wjcc.v10.i7.2322] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/05/2021] [Revised: 11/22/2021] [Accepted: 01/20/2022] [Indexed: 02/06/2023] Open
Abstract
BACKGROUND Gall bladder neuroendocrine tumors (GB-NETs) are rare, accounting for less than 0.5% of all NETs. They usually lack specific symptoms and are difficult to diagnose preoperatively. In most cases, GB-NETs are incidentally found after cholecystectomy for large polyps or cholelithiasis, causing acute or chronic cholecystitis. The coexistence of GB-NET and GB adenocarcinoma is very rare.
CASE SUMMARY We report a case of synchronous but separate GB-NET and adenoma with high-grade dysplasia in a patient who had undergone surgery for a progressively growing GB polypoid lesion. To the best of our knowledge, simultaneous separation of NETs and cancer in the GB has not been reported.
CONCLUSION Coexistent GB carcinoid tumor and adenocarcinoma is rare. A surveillance program is needed for these large GB polyps.
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Affiliation(s)
- Tsung-Hsien Hsiao
- Division of Gastroenterology and Hepatology, Taipei Tzu Chi Hospital, New Taipei City 231, Taiwan
| | - Chao-Chuan Wu
- Department of Surgery, Taipei Tzu Chi Hospital, New Taipei City 231, Taiwan
| | - Hui-Hwa Tseng
- Department of Pathology, Taipei Tzu Chi Hospital, New Taipei City 231, Taiwan
| | - Jiann-Hwa Chen
- Division of Gastroenterology and Hepatology; Department of Internal Medicine, Taipei Tzu Chi Hospital, Buddhist Tzu Chi Medical Foundation, New Taipei City 231, Taiwan
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33
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Dhakre VW, Purushothaman G, Doctor N. Gallbladder Neuroendocrine Tumors: Is There a Need for a Specific Approach? Gastrointest Tumors 2022; 9:5-11. [PMID: 35528747 PMCID: PMC9021648 DOI: 10.1159/000520988] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/17/2021] [Accepted: 11/15/2021] [Indexed: 10/06/2023] Open
Abstract
Neuroendocrine tumors (NETs) of the gallbladder or the biliary tree are rare. Most of the current guidelines and protocols are derived from the experience of managing lung small cell neuroendocrine carcinoma or gastrointestinal NETs. But, the overall outcome of gallbladder NETs (GB-NETs) seems worse than similarly staged lung NETs and adenocarcinoma of the gallbladder. This may be due to its rarity and lack of literature for a focused approach toward its treatment. Hence, the need for a specifically designed approach might help improve results of treatment for these rare tumors. We share our experience of 2 patients with GB-NETs and their 5-year outcome.
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Affiliation(s)
- Vijay Waman Dhakre
- Department of Surgery, Lokmanya Tilak Municipal Medical College and General Hospital, Mumbai, India
| | - Govind Purushothaman
- Department of Surgical Gastroenterology & HPB Surgery, Jaslok Hospital and Research Centre, Mumbai, India
| | - Nilesh Doctor
- Department of Surgical Gastroenterology & HPB Surgery, Jaslok Hospital and Research Centre, Mumbai, India
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34
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Wang W, Yang CX, Yu XZ, Zhang SL, Wang J, Wang J. Clinicopathological characteristics and prognostic factors of patients with primary gallbladder neuroendocrine carcinomas. J Dig Dis 2022; 23:166-173. [PMID: 35187836 DOI: 10.1111/1751-2980.13088] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/24/2021] [Revised: 01/30/2022] [Accepted: 02/14/2022] [Indexed: 02/05/2023]
Abstract
OBJECTIVES Primary gallbladder neuroendocrine carcinomas (GB-NEC) are malignant neoplasms that remained to be studied. In this study we aimed to summarize their clinicopathological characteristics, effective treatment and prognostic factors for patients with GB-NEC. METHODS Patients with GB-NEC admitted to Shanghai Jiao Tong University Affiliated Sixth People's Hospital and Renji Hospital, School of Medicine, Shanghai Jiao Tong University from October 2012 to August 2020 were enrolled. Clinicopathological characteristics of our patients and those reported in previous studies were recorded. The Kaplan-Meier method and univariate and multivariate Cox regression analyses were used for survival analysis. RESULTS Altogether 15 patients from our hospitals and 47 patients from previous studies were included. A total of 55 patients who underwent surgical resection, including R0 and non-R0 resection, had significantly longer overall survival compared with the other seven patients. A univariate analysis indicated that patients aged 60 years or older, with jaundice, carcinoid syndrome, non-R0 resection, and advanced stage were associated with worse survival. A multivariate analysis showed that patients aged 60 years or older, carcinoid syndrome and non-R0 resection, but not lymphadenectomy and adjuvant chemotherapy, were independently related to reduced survival. CONCLUSIONS R0 resection should be the first-line treatment for GB-NEC. Older age, carcinoid syndrome and non-R0 resection are independently associated with reduced survival after surgical resection.
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Affiliation(s)
- Wei Wang
- Department of Hepatobiliary and Pancreatic Surgery, Shanghai Jiao Tong University Affiliated Sixth People's Hospital, Shanghai, China
- Department of Biliary-Pancreatic Surgery, Renji Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai, China
| | - Chuan Xin Yang
- Department of Hepatobiliary and Pancreatic Surgery, Shanghai Jiao Tong University Affiliated Sixth People's Hospital, Shanghai, China
| | - Xin Zhe Yu
- Department of Hepatobiliary and Pancreatic Surgery, Shanghai Jiao Tong University Affiliated Sixth People's Hospital, Shanghai, China
| | - Shi Lei Zhang
- Department of Pathology, Renji Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai, China
| | - Jie Wang
- Department of Hepatobiliary and Pancreatic Surgery, Shanghai Jiao Tong University Affiliated Sixth People's Hospital, Shanghai, China
| | - Jian Wang
- Department of Hepatobiliary and Pancreatic Surgery, Shanghai Jiao Tong University Affiliated Sixth People's Hospital, Shanghai, China
- Department of Biliary-Pancreatic Surgery, Renji Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai, China
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35
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Chandel K, Lal A, Srinivas RK, Chatterjee D, Gupta V. Gallbladder Neuroendocrine Carcinoma: A Case Report with Radiological–Pathological Correlation. JOURNAL OF GASTROINTESTINAL AND ABDOMINAL RADIOLOGY 2022. [DOI: 10.1055/s-0042-1742676] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 10/19/2022] Open
Abstract
AbstractNeuroendocrine tumors (NET) of the biliary system, specifically the gallbladder (GB), are extremely rare, accounting for only 0.2% of all NETs. The majority of GBNETs are discovered by chance during a histological examination of GB samples at autopsy, after cholecystectomy, or after surgery for other suspected biliary pathology. GBNETs are thought to develop from preexisting endocrine cells in the GB's neck or from induced endocrine cells caused by intestinal metaplasia of the body and fundus. We present a case of squamous cell carcinoma of the GB with neuroendocrine differentiation due to its rarity.
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Affiliation(s)
- Karamvir Chandel
- Department of Radiodiagnosis and Imaging, Postgraduate Institute of Medical Education and Research, Chandigarh, India
| | - Anupam Lal
- Department of Radiodiagnosis and Imaging, Postgraduate Institute of Medical Education and Research, Chandigarh, India
| | - Rohit K. Srinivas
- Department of Radiodiagnosis and Imaging, Postgraduate Institute of Medical Education and Research, Chandigarh, India
| | - Debajyoti Chatterjee
- Department of Pathology, Postgraduate Institute of Medical Education and Research, Chandigarh, India
| | - Vikas Gupta
- Department of Surgery, Postgraduate Institute of Medical Education and Research, Chandigarh, India
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36
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Zhang Z, Zhong D, Feng T, Yao Y, Huang X. Mixed Adenoneuroendocrine Carcinoma of the Gallbladder. J Gastrointest Surg 2022; 26:503-506. [PMID: 34668162 DOI: 10.1007/s11605-021-05139-2] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/20/2021] [Accepted: 08/02/2021] [Indexed: 01/31/2023]
Affiliation(s)
- Zilong Zhang
- Department of Hepatobiliary-Pancreatic Surgery, Cell Transplantation Center, Sichuan Provincial People's Hospital, University of Electronic Science and Technology of China, Chengdu, China.,Chinese Academy of Sciences Sichuan Translational Medicine Research Hospital, Chengdu, 610072, China
| | - Deyuan Zhong
- Department of Hepatobiliary-Pancreatic Surgery, Cell Transplantation Center, Sichuan Provincial People's Hospital, University of Electronic Science and Technology of China, Chengdu, China.,Chinese Academy of Sciences Sichuan Translational Medicine Research Hospital, Chengdu, 610072, China
| | - Tianhang Feng
- Department of Hepatobiliary-Pancreatic Surgery, Cell Transplantation Center, Sichuan Provincial People's Hospital, University of Electronic Science and Technology of China, Chengdu, China.,Chinese Academy of Sciences Sichuan Translational Medicine Research Hospital, Chengdu, 610072, China
| | - Yutong Yao
- Department of Hepatobiliary-Pancreatic Surgery, Cell Transplantation Center, Sichuan Provincial People's Hospital, University of Electronic Science and Technology of China, Chengdu, China.,Chinese Academy of Sciences Sichuan Translational Medicine Research Hospital, Chengdu, 610072, China
| | - Xiaolun Huang
- Department of Hepatobiliary-Pancreatic Surgery, Cell Transplantation Center, Sichuan Provincial People's Hospital, University of Electronic Science and Technology of China, Chengdu, China. .,Chinese Academy of Sciences Sichuan Translational Medicine Research Hospital, Chengdu, 610072, China.
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Aashita, Yadav V, Kapoor A, Sharma R. Neuroendocrine tumor gall bladder: A case report and review of literature. JOURNAL OF MEDICAL SCIENCES 2022. [DOI: 10.4103/jmedsci.jmedsci_17_21] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/04/2022] Open
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Wagatsuma K, Akita K, Motoya M, Kimura Y, Sugita S, Hirano T, Kawakami Y, Numata Y, Ishigami K, Masaki Y, Murota A, Shitani M, Akutsu N, Sasaki S, Nakase H. Mixed neuroendocrine non-neuroendocrine neoplasm of the gallbladder complicated by a pancreaticobiliary maljunction of a non-dilated biliary duct: A case report. Medicine (Baltimore) 2021; 100:e27336. [PMID: 34596138 PMCID: PMC8483883 DOI: 10.1097/md.0000000000027336] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/08/2021] [Accepted: 09/09/2021] [Indexed: 01/05/2023] Open
Abstract
RATIONALE Mixed neuroendocrine non-neuroendocrine neoplasm (MiNEN) is a rare tumor. MiNEN of the gallbladder (GB) with pancreaticobiliary maljunction (PMJ) is extremely rare. The origin of MiNEN of the GB remains unknown; the biliary tract normally lacks neuroendocrine cells. MiNEN of the GB has a poor prognosis; because of its rarity, no treatment or management guidelines have been established yet. PATIENT CONCERNS A 47-year-old male presenting with right hypochondrial pain and malaise for 3 months was referred to our hospital for further management. DIAGNOSIS The neuron-specific enolase level was increased. Contrast-enhanced computed tomography revealed a mass of 70 mm in size with unclear boundaries in the liver. The GB was surrounded by this mass, narrowing the lumen of the GB. Many swollen lymph nodes were observed in the hepatoduodenal ligament. Endoscopic retrograde cholangiopancreatography revealed a PMJ with a non-dilated biliary duct. A percutaneous biopsy was performed on the liver mass, and the pathological findings were neuroendocrine carcinoma (NEC) (small cell type). We diagnosed a NEC of the GB, T3N1M0, stage IIIB (Union for International Cancer Control, 7th edition). INTERVENTIONS Because of advanced lymph node metastasis, we considered this tumor difficult to cure solely by surgical intervention. After initial chemotherapy consisting of cisplatin and irinotecan, a marked reduction in both tumor and lymph node sizes enabled conversion surgery. The pathological diagnosis of the resected tumor was MiNEN consisting of NEC and adenocarcinoma. The primary lesion was the adenocarcinoma occupying the luminal side of the GB. As a postsurgical treatment, the patient received additional irradiation therapy to the common hepatic duct and liver stump because of positive surgical margins. OUTCOMES At 13 months postoperatively, computed tomography findings revealed the appearance of a hypervascular liver tumor, and laboratory data showed increased serum neuron-specific enolase levels. Chemotherapy was unsuccessful, leading to the death of the patient 36 months from the date of diagnosis. LESSONS There are several reports on the development of MiNEN of the GB. In our case, a PMJ-related adenocarcinoma of the GB transdifferentiated into NEC. Further accumulation of cases is necessary to establish a treatment strategy for MiNEN of the GB.
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Affiliation(s)
- Kohei Wagatsuma
- Department of Gastroenterology and Hepatology, Sapporo Medical University School of Medicine, Sapporo, Hokkaido, Japan
| | - Kotaro Akita
- Department of Gastroenterology and Hepatology, Sapporo Medical University School of Medicine, Sapporo, Hokkaido, Japan
| | - Masayo Motoya
- Department of Gastroenterology and Hepatology, Sapporo Medical University School of Medicine, Sapporo, Hokkaido, Japan
| | - Yasutoshi Kimura
- Department of Surgery, Surgical Oncology and Science, Sapporo Medical University School of Medicine, Sapporo, Hokkaido, Japan
| | - Shintaro Sugita
- Department of Surgical Pathology, Sapporo Medical University Hospital, Sapporo, Hokkaido, Japan
| | - Takehiro Hirano
- Department of Gastroenterology and Hepatology, Sapporo Medical University School of Medicine, Sapporo, Hokkaido, Japan
| | - Yujiro Kawakami
- Department of Gastroenterology and Hepatology, Sapporo Medical University School of Medicine, Sapporo, Hokkaido, Japan
| | - Yasunao Numata
- Department of Gastroenterology and Hepatology, Sapporo Medical University School of Medicine, Sapporo, Hokkaido, Japan
| | - Keisuke Ishigami
- Department of Gastroenterology and Hepatology, Sapporo Medical University School of Medicine, Sapporo, Hokkaido, Japan
| | - Yoshiharu Masaki
- Department of Gastroenterology and Hepatology, Sapporo Medical University School of Medicine, Sapporo, Hokkaido, Japan
| | - Ayako Murota
- Department of Gastroenterology and Hepatology, Sapporo Medical University School of Medicine, Sapporo, Hokkaido, Japan
| | - Masahiro Shitani
- Department of Gastroenterology and Hepatology, Sapporo Medical University School of Medicine, Sapporo, Hokkaido, Japan
| | - Noriyuki Akutsu
- Department of Gastroenterology and Hepatology, Sapporo Medical University School of Medicine, Sapporo, Hokkaido, Japan
| | - Shigeru Sasaki
- Department of Gastroenterology and Hepatology, Sapporo Medical University School of Medicine, Sapporo, Hokkaido, Japan
| | - Hiroshi Nakase
- Department of Gastroenterology and Hepatology, Sapporo Medical University School of Medicine, Sapporo, Hokkaido, Japan
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Do MY, Jang SI, Kang HP, Kim EJ, Lee KJ, Park GE, Lee SJ, Lee DK, Woo SM, Cho JH. Comparison of the Clinical Features and Outcomes of Gallbladder Neuroendocrine Carcinoma with Those of Adenocarcinoma: A Propensity Score-Matched Analysis. Cancers (Basel) 2021; 13:cancers13184713. [PMID: 34572940 PMCID: PMC8471353 DOI: 10.3390/cancers13184713] [Citation(s) in RCA: 9] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/16/2021] [Revised: 09/16/2021] [Accepted: 09/18/2021] [Indexed: 12/14/2022] Open
Abstract
Simple Summary Neuroendocrine neoplasms (NENs) of the gallbladder (GB) are extremely rare. We aimed to compare the clinical features of GB-NENs with those of adenocarcinomas (ADCs) of the GB. Among 21 patients with GB-NENs, 20 were diagnosed with poorly differentiated small-cell neuroendocrine carcinoma (NEC), and 1 patient had large-cell NEC. At initial presentation, all patients had advanced stages of cancer, with extensive local extension and/or distant metastasis. Nine patients with GB-NEC who underwent surgical resection had a significantly better progression-free survival (PFS) than those who did not undergo surgery. After a propensity score matching with a 1:1 ratio using the American Joint Committee on Cancer (AJCC) stage, age, sex, and operation status, there was no difference in the overall survival or PFS between AJCC stage-matched patients with GB-NEC or GB-ADC. In conclusion, GB-NEC is difficult to diagnose early and has a prognosis similar to that of GB-ADC. Abstract Neuroendocrine neoplasms (NENs) of the gallbladder (GB) are extremely rare. We aimed to compare the clinical features, disease progression, management, and prognosis of patients with GB-NENs with those of patients with GB-adenocarcinomas (ADCs). A total of 21 patients with GB-NENs and 206 patients with GB-ADCs, treated at three tertiary medical centers between January 2010 and December 2020, were enrolled. Of the 21 patients with GB-NENs, 20 were diagnosed with poorly differentiated small-cell neuroendocrine carcinomas (NECs), and 1 patient had large-cell NEC. All patients presented with advanced stages of cancer with extensive local extension and/or distant metastasis and non-specific symptoms. Tumor-node-metastasis stage IIIB and IV (A/B) tumors were found in 6 and 15 (1/14) patients, respectively. Nine patients with GB-NEC who underwent surgical resection had a significantly better progression-free survival (PFS) than those who did not undergo surgery. After a propensity score matching with a 1:1 ratio using the American Joint Committee on Cancer stage, age, sex, and operation status, 19 pairs of patients were included. Compared with stage-matched patients with GB-ADC, patients with GB-NEC had similar overall survival and PFS. However, as GB-NEC is rarely diagnosed early, further studies investigating methods for the early diagnosis and improvement in the survival of patients with GB-NEC are needed.
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Affiliation(s)
- Min-Young Do
- Department of Internal Medicine, Gangnam Severance Hospital, Yonsei University College of Medicine, Seoul 06273, Korea; (M.-Y.D.); (S.-I.J.); (D.-K.L.)
| | - Sung-Ill Jang
- Department of Internal Medicine, Gangnam Severance Hospital, Yonsei University College of Medicine, Seoul 06273, Korea; (M.-Y.D.); (S.-I.J.); (D.-K.L.)
| | - Hua-Pyong Kang
- Department of Internal Medicine, Gachon University Gil Medical Center, Incheon 21565, Korea; (H.-P.K.); (E.-J.K.)
| | - Eui-Joo Kim
- Department of Internal Medicine, Gachon University Gil Medical Center, Incheon 21565, Korea; (H.-P.K.); (E.-J.K.)
| | - Kyong-Joo Lee
- Department of Internal Medicine, Yonsei University Wonju College of Medicine, Wonju 26426, Korea;
| | - Go-Eun Park
- Biostatistics Collaboration Unit, Yonsei University College of Medicine, Seoul 06273, Korea; (G.-E.P.); (S.-J.L.)
| | - Su-Jee Lee
- Biostatistics Collaboration Unit, Yonsei University College of Medicine, Seoul 06273, Korea; (G.-E.P.); (S.-J.L.)
| | - Dong-Ki Lee
- Department of Internal Medicine, Gangnam Severance Hospital, Yonsei University College of Medicine, Seoul 06273, Korea; (M.-Y.D.); (S.-I.J.); (D.-K.L.)
| | - Sang-Myung Woo
- Center for Liver and Pancreatobiliary Cancer, National Cancer Center, Goyang 10408, Korea
- Correspondence: (S.-M.W.); (J.-H.C.)
| | - Jae-Hee Cho
- Department of Internal Medicine, Gangnam Severance Hospital, Yonsei University College of Medicine, Seoul 06273, Korea; (M.-Y.D.); (S.-I.J.); (D.-K.L.)
- Correspondence: (S.-M.W.); (J.-H.C.)
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Zhou S, Jiang S, Chen W, Yin H, Dong L, Zhao H, Han S, He X. Biliary Neuroendocrine Neoplasms: Analysis of Prognostic Factors and Development and Validation of a Nomogram. Front Oncol 2021; 11:654439. [PMID: 34350109 PMCID: PMC8327779 DOI: 10.3389/fonc.2021.654439] [Citation(s) in RCA: 6] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/29/2021] [Accepted: 07/05/2021] [Indexed: 12/26/2022] Open
Abstract
Background For this study, we explored the prognostic profiles of biliary neuroendocrine neoplasms (NENs) patients and identified factors related to prognosis. Further, we developed and validated an effective nomogram to predict the overall survival (OS) of individual patients with biliary NENs. Methods We included a total of 446 biliary NENs patients from the SEER database. We used Kaplan-Meier curves to determine survival time. We employed univariate and multivariate Cox analyses to estimate hazard ratios to identify prognostic factors. We constructed a predictive nomogram based on the results of the multivariate analyses. In addition, we included 28 biliary NENs cases from our center as an external validation cohort. Results The median survival time of biliary NENs from the SEER database was 31 months, and the value of gallbladder NENs (23 months) was significantly shorter than that of the bile duct (45 months) and ampulla of Vater (33.5 months, p=0.023). Multivariate Cox analyses indicated that age, tumor size, pathological classification, SEER stage, and surgery were independent variables associated with survival. The constructed prognostic nomogram demonstrated good calibration and discrimination C-index values of 0.783 and 0.795 in the training and validation dataset, respectively. Conclusion Age, tumor size, pathological classification, SEER stage, and surgery were predictors for the survival of biliary NENs. We developed a nomogram that could determine the 3-year and 5-year OS rates. Through validation of our central database, the novel nomogram is a useful tool for clinicians in estimating individual survival among biliary NENs patients.
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Affiliation(s)
- Shengnan Zhou
- General Surgery Department, Peking Union Medical College Hospital, China Academy of Medical Science & Peking Union Medical College, Beijing, China
| | - Shitao Jiang
- Liver Surgery Department, Peking Union Medical College Hospital, China Academy of Medical Science & Peking Union Medical College, Beijing, China
| | - Weijie Chen
- General Surgery Department, Peking Union Medical College Hospital, China Academy of Medical Science & Peking Union Medical College, Beijing, China
| | - Haixin Yin
- General Surgery Department, Peking Union Medical College Hospital, China Academy of Medical Science & Peking Union Medical College, Beijing, China
| | - Liangbo Dong
- General Surgery Department, Peking Union Medical College Hospital, China Academy of Medical Science & Peking Union Medical College, Beijing, China
| | - Hao Zhao
- General Surgery Department, Peking Union Medical College Hospital, China Academy of Medical Science & Peking Union Medical College, Beijing, China
| | - Shaoqi Han
- General Surgery Department, Peking Union Medical College Hospital, China Academy of Medical Science & Peking Union Medical College, Beijing, China
| | - Xiaodong He
- General Surgery Department, Peking Union Medical College Hospital, China Academy of Medical Science & Peking Union Medical College, Beijing, China
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Neuroendocrine neoplasms of the gallbladder: early detection and surgery is key to improved outcome. Langenbecks Arch Surg 2021; 407:197-206. [PMID: 34236488 DOI: 10.1007/s00423-021-02256-z] [Citation(s) in RCA: 6] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/26/2020] [Accepted: 06/27/2021] [Indexed: 02/07/2023]
Abstract
PURPOSE Neuroendocrine neoplasms (NENs) of the gallbladder are very rare. As a result, the classification of pathologic specimens from gallbladder NENs, currently classified as gallbladder neuroendocrine tumors (GB-NETs) and carcinomas (GB-NECs), is inconsistent and makes nomenclature, classification, and management difficult. Our study aims to evaluate the epidemiological trend, tumor biology, and outcomes of GB-NET and GB-NEC over the last 5 decades. METHODS This is a retrospective analysis of the SEER database from 1973 to 2016. The epidemiological trend was analyzed using the age-adjusted Joinpoint regression analysis. Survival was assessed with Kaplan-Meier analysis and Cox regression was used to assess predictors of poor survival. RESULTS A total of 482 patients with GB-NEN were identified. Mean age at diagnosis was 65.2 ± 14.3 years. Females outnumbered males (65.6% vs. 34.4%). The Joinpoint nationwide trend analysis showed a 7% increase per year from 1973 to 2016. The mean survival time after diagnosis of GB-NEN was 37.11 ± 55.3 months. The most common pattern of nodal distribution was N0 (50.2%) followed by N1 (30.9%) and N2 (19.2%). Advanced tumor spread (into the liver, regional, and distant metastasis) was seen in 60.3% of patients. Patients who underwent surgery had a significant survival advantage (111.0 ± 8.3 vs. 8.3 ± 1.2 months, p < 0.01). Cox regression analysis showed advanced age (p < 0.01), tumor stage (P < 0.01), tumor extension (p < 0.01), and histopathologic grade (p < 0.01) were associated with higher mortality. CONCLUSION Gallbladder NENs are a rare histopathological variant of gallbladder cancer that is showing a rising incidence in the USA. In addition to tumor staging, surgical resection significantly impacts patient survival, when patients are able to undergo surgery irrespective of tumor staging. Advanced age, tumor extension, and histopathological grade of the tumor were associated with higher mortality.
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Dhali A, Ray S, Dhali GK. Primary Small Cell Neuroendocrine Tumour of Gallbladder Presenting as Pyrexia of Unknown Origin. Cureus 2021; 13:e15874. [PMID: 34327099 PMCID: PMC8302456 DOI: 10.7759/cureus.15874] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 06/23/2021] [Indexed: 12/22/2022] Open
Abstract
Herein, we report a case of primary small cell neuroendocrine tumor (NET) of the gallbladder in a 45-year-old female who presented with complaints of fever accompanied by abdominal pain on the right upper quadrant for one month. Contrast-enhanced computed tomography abdomen showed a large gallbladder mass. It was infiltrating the segments IVa, IVb, and V of the liver. Ultrasound-guided fine-needle-aspiration-cytology was performed. Based on preoperative pathological and immunohistochemical investigations, it was diagnosed to be a primary small cell NET of the gallbladder. The patient underwent radical cholecystectomy after three cycles of cisplatin-based neoadjuvant chemotherapy. She had an uneventful recovery and received adjuvant chemo-radiotherapy. The patient was well at the 18-month follow-up.
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Affiliation(s)
- Arkadeep Dhali
- Department of Gastrointestinal Surgery, School of Digestive and Liver Diseases, Institute of Postgraduate Medical Education and Research, Kolkata, IND
| | - Sukanta Ray
- Department of Gastrointestinal Surgery, School of Digestive and Liver Diseases, Institute of Postgraduate Medical Education and Research, Kolkata, IND
| | - Gopal Krishna Dhali
- Department of Gastroenterology, School of Digestive and Liver Diseases, Institute of Postgraduate Medical Education and Research, Kolkata, IND
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Terai T, Nakagawa K, Nakamura K, Doi S, Morita K, Ohbayashi C, Sho M. Neuroendocrine tumor arising from the greater omentum treated with laparoscopic tumor resection: a case report. Surg Case Rep 2021; 7:132. [PMID: 34061282 PMCID: PMC8167924 DOI: 10.1186/s40792-021-01217-4] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/17/2021] [Accepted: 05/24/2021] [Indexed: 11/16/2022] Open
Abstract
Background Primary omental tumors are extremely rare. Herein, we report the first case of a primary omental neuroendocrine tumor (NET). Case presentation A 59-year-old woman was referred to our hospital for the treatment of an 18-mm tumor located at the ventral side of the duodenum. No other tumor was detected. The preoperative imaging diagnosis was omental tumor. A laparoscopic tumor resection was performed. Histopathological examination revealed that the tumor consisted of cuboidal cells with eosinophilic, granular cytoplasm showing trabecular or ribbon architecture. No other component was seen. The mitotic count was of 5 per 10 high-power fields. Immunohistochemical staining was positive for chromogranin A, synaptophysin, and CD56. Her Ki-67 index was 5%. These results led to the diagnosis of grade 2 omental NET. The patient was discharged on the 3rd postoperative day without any complications and did not develop any recurrence for 3 years. Conclusions We encountered a very rare case of omental NET. Complete resection is recommended with minimally invasive surgery for the diagnosis of NET. Supplementary Information The online version contains supplementary material available at 10.1186/s40792-021-01217-4.
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Affiliation(s)
- Taichi Terai
- Department of Surgery, Nara Medical University, 840 Shijo-cho, Kashihara, Nara, 634-8522, Japan.
| | - Kenji Nakagawa
- Department of Surgery, Nara Medical University, 840 Shijo-cho, Kashihara, Nara, 634-8522, Japan
| | - Kota Nakamura
- Department of Surgery, Nara Medical University, 840 Shijo-cho, Kashihara, Nara, 634-8522, Japan
| | - Shunsuke Doi
- Department of Surgery, Nara Medical University, 840 Shijo-cho, Kashihara, Nara, 634-8522, Japan
| | - Kohei Morita
- Department of Diagnostic Pathology, Nara Medical University, 840 Shijo-cho, Kashihara, Nara, 634-8522, Japan
| | - Chiho Ohbayashi
- Department of Diagnostic Pathology, Nara Medical University, 840 Shijo-cho, Kashihara, Nara, 634-8522, Japan
| | - Masayuki Sho
- Department of Surgery, Nara Medical University, 840 Shijo-cho, Kashihara, Nara, 634-8522, Japan
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Wang P, Chen J, Jiang Y, Jia C, Pang J, Wang S, Chang X. Neuroendocrine Neoplasms of the Gallbladder: A Clinicopathological Analysis of 13 Patients and a Review of the Literature. Gastroenterol Res Pract 2021; 2021:5592525. [PMID: 34122537 PMCID: PMC8166508 DOI: 10.1155/2021/5592525] [Citation(s) in RCA: 9] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/28/2021] [Accepted: 05/10/2021] [Indexed: 12/16/2022] Open
Abstract
OBJECTIVES Mixed neuroendocrine-non-neuroendocrine neoplasms (MiNENs) are rare gallbladder neuroendocrine neoplasms (GB-NENs). This study is aimed at investigating the clinicopathological features of GB-NENs and identifying prognostic factors related to overall survival (OS) of GB-MiNENs. METHODS The clinical data and pathological features of 13 patients with GB-NENs in our hospital were retrospectively reviewed. Additionally, 41 GB-MiNENs cases reported in English literature were reviewed and survival analysis was performed. RESULTS The mean age of thirteen patients (6 males and 7 females) with GB-NENs was 57.2 years (range: 35-75 years). Two patients were diagnosed with NET grade 1 (G1), two patients with NEC (large cell/small cell = 1/1), and nine patients with MiNENs. Of these 9 patients with MiNENs, 8 had composite tumors and 1 had amphicrine carcinoma. Microscopically, the adenocarcinoma component was located in the surface mucosa, and the neuroendocrine component was in the area of deep invasion, liver infiltration, and lymph node metastasis. Total analysis of 41 GB-MiNENs showed that patients were mainly elderly women (female/male ratio, 2.4 : 1.0; median age, 60 years). Kaplan-Meier's analysis demonstrated that liver metastasis and TNM stage III-IV were associated with decreased OS (P < 0.05), whereas age, sex, tumor size, grade of the neuroendocrine component, lymph node metastasis, and adjuvant chemotherapy were not significantly prognostic indicators of OS. Multivariate analysis identified liver metastasis (hazard ratio = 4.262, 95%confidence interval = 1.066-17.044, P = 0.040) as an independent unfavorable prognostic factor. CONCLUSIONS GB-MiNENs were the most common type of GB-NENs in our case series, and neuroendocrine components exhibited more aggressive lymph node metastasis and local invasion than adenocarcinoma. Liver metastasis was a poor prognostic indicator in GB-MiNENs patients.
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Affiliation(s)
- Pengyan Wang
- Department of Pathology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Tsinghua University, Beijing 100730, China
| | - Jingci Chen
- Department of Pathology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Tsinghua University, Beijing 100730, China
| | - Ying Jiang
- Department of Pathology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Tsinghua University, Beijing 100730, China
| | - Congwei Jia
- Department of Pathology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Tsinghua University, Beijing 100730, China
| | - Junyi Pang
- Department of Pathology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Tsinghua University, Beijing 100730, China
| | - Shan Wang
- Department of Pathology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Tsinghua University, Beijing 100730, China
| | - Xiaoyan Chang
- Department of Pathology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Tsinghua University, Beijing 100730, China
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Chablou M, Mabrouk Y, Maamar K, Jabi R, Bouziane M. Neuroendocrine carcinoma of the gallbladder concomitant with adenocarcinoma of the sigmoid colon: A rare case report. Ann Med Surg (Lond) 2021; 66:102359. [PMID: 34026109 PMCID: PMC8131395 DOI: 10.1016/j.amsu.2021.102359] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/09/2021] [Revised: 04/15/2021] [Accepted: 04/25/2021] [Indexed: 11/26/2022] Open
Abstract
Introduction and importance Neuroendocrine carcinoma of the gallbladder is rare and aggressive, its diagnosis is based on pathologic and immunohistochemical examination, currently there is no standard treatment for this tumor. Its concomitant occurrence with adenocarcinoma of the sigmoid colon is exceptional. Case presentation This case report describes a rare case of neuroendocrine carcinoma of the gallbladder that occurred concomitantly with sigmoid colon adenocarcinoma. The diagnosis of neuroendocrine carcinoma was established postoperatively by pathological and immunohistochemical examination. The biopsy of the colonic process confirmed the preoperative diagnosis. A laparotomy found a vesicular fundus process, peri-hilar adenopathy, metastasis of the liver segment V, and a sigmoid colon process. The patient underwent a cholecystectomy extended to segments IVb and V, with lymph node dissection and sigmoid colectomy. The postoperative follow-up was uneventful, and the length of hospitalization was seven days. The patient is currently undergoing adjuvant chemotherapy. Discussion Neuroendocrine carcinoma of the gallbladder is extremely rare, with few case reports, its occurrence concomitantly with adenocarcinoma of the sigmoid colon is exceptional. Histology and immunohistochemistry confirm the diagnosis, surgery is essential in the management of patients affected by this tumor. The presence of synchronous metastases causes doubt about their primitive, hence the primordial place of surgery and histological examination to adopt adequate management of the patients. Conclusion We underline the rarity of this neuroendocrine tumor and the importance of histology and surgery in its management.
Neuroendocrine carcinoma of the gallbladder is a rare and aggressive tumor usually diagnosed at an advanced stage. The patient presented with a vague abdominal pain and bleeding per rectum. The final diagnosis is made upon histological and immunohistochemical examination. The concomitant occurrence neuroendocrine neoplasm of the gallbladder and adenocarcinoma of the sigmoid is exceptional.
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Affiliation(s)
- Mohamed Chablou
- General Surgery Department, Mohammed VI University Hospital/Faculty of Medicine and Pharmacy, Mohammed First University, Oujda, Morocco
| | - Yassine Mabrouk
- General Surgery Department, Mohammed VI University Hospital/Faculty of Medicine and Pharmacy, Mohammed First University, Oujda, Morocco
| | - Khalil Maamar
- General Surgery Department, Mohammed VI University Hospital/Faculty of Medicine and Pharmacy, Mohammed First University, Oujda, Morocco
| | - Rachid Jabi
- General Surgery Department, Mohammed VI University Hospital/Faculty of Medicine and Pharmacy, Mohammed First University, Oujda, Morocco
| | - Mohammed Bouziane
- General Surgery Department, Mohammed VI University Hospital/Faculty of Medicine and Pharmacy, Mohammed First University, Oujda, Morocco
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Chu H, Shi Y, Liu J, Huang D, Zhang J, Dou C. Update in clinical management for gallbladder neuroendocrine carcinoma. Medicine (Baltimore) 2021; 100:e25449. [PMID: 33832150 PMCID: PMC8036038 DOI: 10.1097/md.0000000000025449] [Citation(s) in RCA: 13] [Impact Index Per Article: 3.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/12/2020] [Accepted: 03/10/2021] [Indexed: 01/05/2023] Open
Abstract
BACKGROUND Gallbladder neuroendocrine carcinoma (GB-NEC) is rare and there are few reports at present. We sought to review the current knowledge of GB-NEC and provide recommendations for clinical management. METHODS A systemic literature research was conducted in the websites of Pubmed, Medline, Web of Science, CNKI, Wanfang Data using the keywords including gallbladder combined with neuroendocrine carcinoma or neuroendocrine tumor or neuroendocrine neoplasm. Two reviewers independently screened the articles by reading the title, abstract and full-text. RESULTS In computed tomography (CT) and magnetic resonance imaging (MRI) examination, a well-defined margin, gallbladder replacing type with larger hepatic and lymphatic metastases could be helpful for differential diagnosis of GB-NEC and gallbladder adenocarcinoma (GB-ADC). Older age, unmarried status, large tumor size (>5 cm), positive margins, and distant Surveillance, Epidemiology and End result (SEER) stage are independently associated with poor survival. Surgical resection remains as the preferred and primary treatment. The potential survival benefit of lymphadenectomy for patients remains controversial. Platinum-based postoperative adjuvant chemotherapy may improve the survival. The efficacy of other treatments including immunotherapy, targeted therapy and somatostatin analogue needs further investigation. CONCLUSION Typical imaging features could be helpful for preoperative diagnosis. Age, margin status, tumor size, marital status, histopathologic subtype and SEER stage may be independent predictors for the survival. Remarkable advances regarding the treatment for GB-NEC have been achieved in recent years. Further studies are needed to investigate the survival benefit of lymphadenectomy for patients with GB-NEC.
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Affiliation(s)
- Hongwu Chu
- Hepatobiliary and Pancreatic Surgery, Zhejiang Provincial People's Hospital, Hangzhou Medical College, Hangzhou
- Qingdao University, Qingdao
| | - Ying Shi
- Obstetrics and Gynecology, Zhejiang Provincial People's Hospital, Hangzhou Medical College, Hangzhou, China
| | - Junwei Liu
- Hepatobiliary and Pancreatic Surgery, Zhejiang Provincial People's Hospital, Hangzhou Medical College, Hangzhou
| | - Dongsheng Huang
- Hepatobiliary and Pancreatic Surgery, Zhejiang Provincial People's Hospital, Hangzhou Medical College, Hangzhou
| | - Jungang Zhang
- Hepatobiliary and Pancreatic Surgery, Zhejiang Provincial People's Hospital, Hangzhou Medical College, Hangzhou
| | - Changwei Dou
- Hepatobiliary and Pancreatic Surgery, Zhejiang Provincial People's Hospital, Hangzhou Medical College, Hangzhou
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Liu F, Li Y, Ying D, Qiu S, He Y, Li M, Liu Y, Zhang Y, Zhu Q, Hu Y, Liu L, Li G, Pan W, Jin W, Mu J, Cao Y, Liu Y. Whole-exome mutational landscape of neuroendocrine carcinomas of the gallbladder. Signal Transduct Target Ther 2021; 6:55. [PMID: 33563892 PMCID: PMC7873252 DOI: 10.1038/s41392-020-00412-3] [Citation(s) in RCA: 10] [Impact Index Per Article: 2.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/07/2020] [Revised: 10/14/2020] [Accepted: 10/28/2020] [Indexed: 12/23/2022] Open
Abstract
Neuroendocrine carcinoma (NEC) of the gallbladder (GB-NEC) is a rare but extremely malignant subtype of gallbladder cancer (GBC). The genetic and molecular signatures of GB-NEC are poorly understood; thus, molecular targeting is currently unavailable. In the present study, we applied whole-exome sequencing (WES) technology to detect gene mutations and predicted somatic single-nucleotide variants (SNVs) in 15 cases of GB-NEC and 22 cases of general GBC. In 15 GB-NECs, the C > T mutation was predominant among the 6 types of SNVs. TP53 showed the highest mutation frequency (73%, 11/15). Compared with neuroendocrine carcinomas of other organs, significantly mutated genes (SMGs) in GB-NECs were more similar to those in pulmonary large-cell neuroendocrine carcinomas (LCNECs), with driver roles for TP53 and RB1. In the COSMIC database of cancer-related genes, 211 genes were mutated. Strikingly, RB1 (4/15, 27%) and NAB2 (3/15, 20%) mutations were found specifically in GB-NECs; in contrast, mutations in 29 genes, including ERBB2 and ERBB3, were identified exclusively in GBC. Mutations in RB1 and NAB2 were significantly related to downregulation of the RB1 and NAB2 proteins, respectively, according to immunohistochemical (IHC) data (p values = 0.0453 and 0.0303). Clinically actionable genes indicated 23 mutated genes, including ALK, BRCA1, and BRCA2. In addition, potential somatic SNVs predicted by ISOWN and SomVarIUS constituted 6 primary COSMIC mutation signatures (1, 3, 30, 6, 7, and 13) in GB-NEC. Genes carrying somatic SNVs were enriched mainly in oncogenic signaling pathways involving the Notch, WNT, Hippo, and RTK-RAS pathways. In summary, we have systematically identified the mutation landscape of GB-NEC, and these findings may provide mechanistic insights into the specific pathogenesis of this deadly disease.
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Affiliation(s)
- Fatao Liu
- Department of General Surgery, Xinhua Hospital Affiliated to Shanghai Jiao Tong University School of Medicine, Yangpu District, Shanghai, 200092, China
- Shanghai Key Laboratory of Biliary Tract Disease, Yangpu District, Shanghai, 200092, China
- State Key Laboratory of Oncogenes and Related Genes, Shanghai, China
- Shanghai Research Center of Biliary Tract Disease, Yangpu District, Shanghai, 200092, China
| | - Yongsheng Li
- Shanghai Key Laboratory of Biliary Tract Disease, Yangpu District, Shanghai, 200092, China
- State Key Laboratory of Oncogenes and Related Genes, Shanghai, China
- Shanghai Research Center of Biliary Tract Disease, Yangpu District, Shanghai, 200092, China
- Department of Biliary-Pancreatic Surgery, Renji Hospital Affiliated to Shanghai Jiao Tong University School of Medicine, Shanghai, China
| | - Dongjian Ying
- Department of Minimal Invasive Surgery, Ningbo Medical Center Lihuili Hospital, Ningbo, Zhejiang, 315040, China
| | - Shimei Qiu
- Department of General Surgery, Xinhua Hospital Affiliated to Shanghai Jiao Tong University School of Medicine, Yangpu District, Shanghai, 200092, China
- Shanghai Key Laboratory of Biliary Tract Disease, Yangpu District, Shanghai, 200092, China
- State Key Laboratory of Oncogenes and Related Genes, Shanghai, China
- Shanghai Research Center of Biliary Tract Disease, Yangpu District, Shanghai, 200092, China
| | - Yong He
- Department of hepatopancreatobiliary surgery, Ganzhou hospital affiliated to Nanchang university, Jiangxi, 341000, China
| | - Maolan Li
- Department of General Surgery, Xinhua Hospital Affiliated to Shanghai Jiao Tong University School of Medicine, Yangpu District, Shanghai, 200092, China
- Shanghai Key Laboratory of Biliary Tract Disease, Yangpu District, Shanghai, 200092, China
- Shanghai Research Center of Biliary Tract Disease, Yangpu District, Shanghai, 200092, China
| | - Yun Liu
- Shanghai Key Laboratory of Biliary Tract Disease, Yangpu District, Shanghai, 200092, China
- State Key Laboratory of Oncogenes and Related Genes, Shanghai, China
- Shanghai Research Center of Biliary Tract Disease, Yangpu District, Shanghai, 200092, China
- Department of Biliary-Pancreatic Surgery, Renji Hospital Affiliated to Shanghai Jiao Tong University School of Medicine, Shanghai, China
| | - Yijian Zhang
- Department of General Surgery, Xinhua Hospital Affiliated to Shanghai Jiao Tong University School of Medicine, Yangpu District, Shanghai, 200092, China
- Shanghai Key Laboratory of Biliary Tract Disease, Yangpu District, Shanghai, 200092, China
- State Key Laboratory of Oncogenes and Related Genes, Shanghai, China
- Shanghai Research Center of Biliary Tract Disease, Yangpu District, Shanghai, 200092, China
| | - Qin Zhu
- Department of General Surgery, Xinhua Hospital Affiliated to Shanghai Jiao Tong University School of Medicine, Yangpu District, Shanghai, 200092, China
- Shanghai Key Laboratory of Biliary Tract Disease, Yangpu District, Shanghai, 200092, China
- State Key Laboratory of Oncogenes and Related Genes, Shanghai, China
- Shanghai Research Center of Biliary Tract Disease, Yangpu District, Shanghai, 200092, China
| | - Yunping Hu
- Department of General Surgery, Xinhua Hospital Affiliated to Shanghai Jiao Tong University School of Medicine, Yangpu District, Shanghai, 200092, China
- Shanghai Key Laboratory of Biliary Tract Disease, Yangpu District, Shanghai, 200092, China
- State Key Laboratory of Oncogenes and Related Genes, Shanghai, China
- Shanghai Research Center of Biliary Tract Disease, Yangpu District, Shanghai, 200092, China
| | - Liguo Liu
- Department of General Surgery, Xinhua Hospital Affiliated to Shanghai Jiao Tong University School of Medicine, Yangpu District, Shanghai, 200092, China
- Shanghai Key Laboratory of Biliary Tract Disease, Yangpu District, Shanghai, 200092, China
- State Key Laboratory of Oncogenes and Related Genes, Shanghai, China
- Shanghai Research Center of Biliary Tract Disease, Yangpu District, Shanghai, 200092, China
- Department of Biliary-Pancreatic Surgery, Renji Hospital Affiliated to Shanghai Jiao Tong University School of Medicine, Shanghai, China
| | - Guoqiang Li
- Department of General Surgery, Xinhua Hospital Affiliated to Shanghai Jiao Tong University School of Medicine, Yangpu District, Shanghai, 200092, China
- Shanghai Key Laboratory of Biliary Tract Disease, Yangpu District, Shanghai, 200092, China
- State Key Laboratory of Oncogenes and Related Genes, Shanghai, China
- Shanghai Research Center of Biliary Tract Disease, Yangpu District, Shanghai, 200092, China
- Department of Biliary-Pancreatic Surgery, Renji Hospital Affiliated to Shanghai Jiao Tong University School of Medicine, Shanghai, China
| | - Weihua Pan
- Department of Pediatric Surgery, Xinhua Hospital Affiliated to Shanghai Jiao Tong University School of Medicine, Yangpu District, Shanghai, 200092, China
- Information and Big Data Center, Xinhua Hospital Affiliated to Shanghai Jiao Tong University School of Medicine, Yangpu District, Shanghai, 200092, China
| | - Wei Jin
- Information and Big Data Center, Xinhua Hospital Affiliated to Shanghai Jiao Tong University School of Medicine, Yangpu District, Shanghai, 200092, China
| | - Jiasheng Mu
- Department of General Surgery, Xinhua Hospital Affiliated to Shanghai Jiao Tong University School of Medicine, Yangpu District, Shanghai, 200092, China.
- Information and Big Data Center, Xinhua Hospital Affiliated to Shanghai Jiao Tong University School of Medicine, Yangpu District, Shanghai, 200092, China.
| | - Yang Cao
- Department of Surgery, Second Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou, Zhejiang, 310009, China.
- Department of Gastric Surgery, Cancer Hospital of the University of Chinese Academy of Sciences, Hangzhou, Zhejiang, 310022, China.
| | - Yingbin Liu
- Shanghai Key Laboratory of Biliary Tract Disease, Yangpu District, Shanghai, 200092, China.
- State Key Laboratory of Oncogenes and Related Genes, Shanghai, China.
- Shanghai Research Center of Biliary Tract Disease, Yangpu District, Shanghai, 200092, China.
- Department of Biliary-Pancreatic Surgery, Renji Hospital Affiliated to Shanghai Jiao Tong University School of Medicine, Shanghai, China.
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Luchini C, Pelosi G, Scarpa A, Mattiolo P, Marchiori D, Maragliano R, Sessa F, Uccella S. Neuroendocrine neoplasms of the biliary tree, liver and pancreas: a pathological approach. Pathologica 2021; 113:28-38. [PMID: 33686308 PMCID: PMC8138696 DOI: 10.32074/1591-951x-231] [Citation(s) in RCA: 22] [Impact Index Per Article: 5.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/23/2020] [Accepted: 11/30/2020] [Indexed: 12/14/2022] Open
Abstract
Neuroendocrine neoplasms of the pancreatobiliary tract and liver are a heterogeneous group that encompass a spectrum of entities with distinct morphological, biological and clinical features. Although in the various anatomical sub-sites of this region they show specific characteristics, these tumors, as a whole, share several etiological and clinical aspects. This review systematically addresses NENs arising in the extrahepatic bile ducts, gallbladder, liver and pancreas, with the principal aim of pinpointing essential diagnostic and classification issues. In addition, the section on hepatic NENs has been expanded to include metastatic disease of unknown primary site.
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Affiliation(s)
- Claudio Luchini
- Department of Diagnostics and Public Health, Section of Pathology, University and Hospital Trust of Verona, Italy
| | - Giuseppe Pelosi
- Department of Oncology and Hemato-Oncology, University of Milan, Italy
- Inter-Hospital Pathology Division, IRCCS MultiMedica, Milan, Italy
| | - Aldo Scarpa
- Department of Diagnostics and Public Health, Section of Pathology, University and Hospital Trust of Verona, Italy
- ARC-NET Research Centre, University of Verona, Italy
| | - Paola Mattiolo
- Department of Diagnostics and Public Health, Section of Pathology, University and Hospital Trust of Verona, Italy
| | - Deborah Marchiori
- Department of Medicine and Surgery, Unit of Pathology, University of Insubria, Varese, Italy
| | - Roberta Maragliano
- Department of Medicine and Surgery, Unit of Pathology, University of Insubria, Varese, Italy
| | - Fausto Sessa
- Department of Medicine and Surgery, Unit of Pathology, University of Insubria, Varese, Italy
| | - Silvia Uccella
- Department of Medicine and Surgery, Unit of Pathology, University of Insubria, Varese, Italy
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Clear cell neuroendocrine tumor in the gallbladder diagnosed as a benign polyp preoperatively: a case report. World J Surg Oncol 2021; 19:3. [PMID: 33388069 PMCID: PMC7778816 DOI: 10.1186/s12957-020-02104-2] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/01/2020] [Accepted: 12/02/2020] [Indexed: 11/10/2022] Open
Abstract
Background Gallbladder neuroendocrine neoplasm is a rare disease that is divided into neuroendocrine tumors (NETs) and neuroendocrine carcinomas (NECs). Clear cell NETs of the gallbladder are extremely rare. We report the case of a patient with polypoid clear cell NET G1 of the gallbladder who underwent laparoscopic cholecystectomy. Case presentation A 10-mm pedunculated polyp in the gallbladder neck was detected on a follow-up abdominal ultrasound in a 60-year-old man with chronic hepatitis and hepatitis B without medication. Six months later, an abdominal ultrasound revealed that the tumor had enlarged to 12 mm in size. He was asymptomatic and had no abnormalities in other laboratory examinations, including the tumor markers, carcinoembryonic antigen and CA19-9. Abdominal ultrasound showed a 12-mm polyp in the neck of the gallbladder with perfusion and focal thickening of the gallbladder wall. A gallbladder stone was also seen in the fundus. An enhanced computed tomography scan and magnetic resonance imaging revealed a polypoid lesion and gallbladder stone located at the neck of the gallbladder and the fundus, respectively. Malignancy could not be excluded, and hence, a laparoscopic cholecystectomy was performed. Pathologically, a pedunculated polyp (14 × 11 × 15 mm) was observed in the neck of the gallbladder, and the polypoid lesion comprised nests or trabecular growths of clear NET cells in the lamina propria (ENETS: T1N0M0; AJCC: T1aN0M0). Immunohistochemical staining with synaptophysin, chromogranin A, and CD56 was confined to the tumor. The pathological diagnosis was clear cell NET G1 of the gallbladder. Although clear cell NET is often described as a distinct manifestation of von Hippel-Lindau disease (VHL), the patient had no past medical or family history of VHL. Until his one-and-a-half-year follow-up, the patient was doing well and without any signs of recurrence. Conclusion We report an extremely rare case of gallbladder clear cell NET G1. When NET G1 is incidentally identified in a gallbladder surgical specimen, clinical information and pathological findings should be considered as references.
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Randhawa DS, Shoucair S, McCarron E. Silent and formidable foe: neuroendocrine tumours of the gallbladder. BMJ Case Rep 2020; 13:13/12/e237063. [PMID: 33370977 PMCID: PMC7757469 DOI: 10.1136/bcr-2020-237063] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/24/2022] Open
Abstract
Neuroendocrine neoplasms of the gallbladder occur infrequently, with the diagnosis being incidental in most cases. We present a case of an 81-year-old African American woman who initially presented with acute suppurative cholecystitis, found on pathology to have a moderately differentiated infiltrating adenocarcinoma. A partial hepatic resection with periportal lymph node dissection was planned which was subsequently aborted intraoperatively due to the presence of diffuse carcinomatosis. Pathology of the cancerous lesions revealed neuroendocrine carcinoma. Gallbladder neuroendocrine tumours demonstrate no specific clinical features. Given its often late presentation, neuroendocrine tumours of the gallbladder pose a therapeutic and prognostic challenge.
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Affiliation(s)
| | - Sami Shoucair
- Department of Surgery, MedStar Health, Columbia, Maryland, USA
| | - Edward McCarron
- Department of Surgery, MedStar Health, Columbia, Maryland, USA
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