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Kulshrestha A, Jawale D, Sharma A, Bhoj P, Pandagle A. An Unusual Case of Neuroendocrine Carcinoma of the Gastro-Esophageal Junction Complicated by Syndrome of Inappropriate Antidiuretic Hormone Secretion. Cureus 2025; 17:e79807. [PMID: 40161092 PMCID: PMC11955082 DOI: 10.7759/cureus.79807] [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: 02/27/2025] [Indexed: 04/02/2025] Open
Abstract
Cancers of the esophagus tend to be aggressive with a poor prognosis. Neuroendocrine carcinoma (NEC) of the esophagus is very rare and currently does not have any established treatment regimens. This is in part due to its rare prevalence. This case report presents an unusual case of distal esophageal NEC, which was complicated by liver metastasis and paraneoplastic syndrome. A 56-year-old postmenopausal woman came with complaints of weight loss, pain in the epigastric region, and intermittent dysphagia to solids for three months. An endoscopy revealed a 2 cm x 1 cm ulceronodular lesion located at the gastroesophageal junction. The biopsy showed NEC on histological examination, and the immunohistochemistry was positive for multiple neuroendocrine markers. A positron emission tomography (PET) scan revealed a stage IV metastatic disease that had spread to the liver as well as gastrohepatic and peri-gastric lymph nodes. Subsequently, the patient received palliative intent chemotherapy with Etoposide and Cisplatin for four cycles, which resulted in a partial response. Due to this, she was advised four more cycles of chemotherapy, but after two cycles, she developed signs of the syndrome of inappropriate antidiuretic hormone secretion (SIADH). Following management with hypertonic saline, her health deteriorated, resulting in death within one year of diagnosis. NECs of the esophagus tend to be detected at later stages and show a worse prognosis and disease course than squamous cell carcinoma or adenocarcinoma of the esophagus. With improvements in investigation methods, an increase in the detection of NEC in the esophagus can be expected in the future. Early diagnosis can help overall survival and boost quality of life. Further research and clinical trials are needed to assess ideal treatment plans.
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Affiliation(s)
- Asmita Kulshrestha
- Department of Radiation Oncology, Cama and Albless Hospital, Mumbai, IND
| | - Deeksha Jawale
- Department of Radiation Oncology, Cama and Albless Hospital, Mumbai, IND
| | - Ankit Sharma
- Department of Surgical Oncology, Tata Memorial Hospital, Mumbai, IND
| | - Priyanka Bhoj
- Department of Radiation Oncology, Cama and Albless Hospital, Mumbai, IND
| | - Amitabh Pandagle
- Department of Radiation Oncology, Cama and Albless Hospital, Mumbai, IND
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2
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Yang T, Yang P, Li H, Sun M, Zhang J, Li Y, Zhu B. Association of treatment methods and survival in esophageal neuroendocrine carcinoma population of Surveillance, Epidemiology, and End Results database: a retrospective cohort study. Dis Esophagus 2025; 38:doaf008. [PMID: 39950356 DOI: 10.1093/dote/doaf008] [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: 11/06/2024] [Revised: 12/25/2024] [Accepted: 01/27/2025] [Indexed: 05/09/2025]
Abstract
Esophageal neuroendocrine carcinoma (ENEC) is an uncommon and highly aggressive tumor with an obscure etiology. Thus, this study aimed to investigate the best treatment option for patients with ENEC. This study identified patients with ENEC from the Surveillance, Epidemiology, and End Results (SEER) database between 1975 and 2021. Clinicopathological characteristics were analyzed using descriptive statistics, and prognostic factors were determined through Cox regression analysis. Kaplan-Meier survival analysis was used to assess five-year overall survival (OS). Patients were divided into a combined radiotherapy and chemotherapy group and a surgery group based on treatment modality. Propensity score matching (PSM) was performed to balance baseline characteristics between the two groups. A total of 604 participants were included; univariate and multivariate analyses indicated that ENEC patients with localized or regional disease had significantly higher survival rates (HR = 0.38, 95% CI 0.29-0.51, P < 0.001 and HR = 0.56, 95% CI 0.44-0.71, P < 0.001) than those with distant metastases. The combination of radiotherapy and chemotherapy (HR = 0.34, 95% CI 0.25-0.46, P < 0.001) and surgical treatment (HR = 0.43, 95% CI 0.31-0.59, P < 0.001) significantly improved survival. Kaplan-Meier analysis showed significant differences in OS among treatments (P < 0.0001), with the combination therapy and surgery groups having similar rates of 7.04% and 8.77%, respectively, and outperforming other methods. The combination therapy group maintained the best survival rate over time. The synergistic application of radiotherapy and chemotherapy has markedly enhanced the five-year survival rate of patients with ENEC, offering invaluable insights for clinical management strategies.
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Affiliation(s)
- T Yang
- College of Pharmacy, Inner Mongolia Medical University, Hohhot, China
| | - P Yang
- Department of Thoracic Surgery, Peking University Cancer Hospital (Inner Mongolia Campus)/Affiliated Cancer Hospital of Inner Mongolia Medical University, Hohhot, Inner Mongolia Autonomous Region 010050, China
| | - H Li
- Department of Digestive Medicine, Zhungeerqi Central Hospital, Erdos, Inner Mongolia Autonomous Region, China
| | - M Sun
- College of Pharmacy, Inner Mongolia Medical University, Hohhot, China
| | - J Zhang
- College of Pharmacy, Inner Mongolia Medical University, Hohhot, China
| | - Y Li
- Department of Thoracic Surgery, National Cancer Center/National Clinical Research Center for Cancer/Cancer Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing, China
| | - B Zhu
- Department of Pharmacy, Peking University Cancer Hospital (Inner Mongolia Campus)/Affiliated Cancer Hospital of Inner Mongolia Medical University, Hohhot, Inner Mongolia Autonomous Region 010050, China
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3
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Taori K, Kirnake V, Junare PR, Padwale V. An Uncommon Encounter: A Comprehensive Case Report of an Advanced Esophageal Neuroendocrine Carcinoma. Cureus 2024; 16:e53027. [PMID: 38410310 PMCID: PMC10895312 DOI: 10.7759/cureus.53027] [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: 11/08/2023] [Accepted: 01/26/2024] [Indexed: 02/28/2024] Open
Abstract
Esophageal neuroendocrine carcinomas (E-NECs) are rare malignant tumors with unknown etiology and pathogenesis. The aggressive nature of E-NECs coupled with a tendency to metastasize and no available treatment guidelines lead to poor prognosis. Here, we report a case of a 65-year-old, previously healthy female who presented with difficulty in swallowing solids, burning sensation over the epigastric region, weight loss (>10%), and altered bowel habits for the last three months. Contrast-enhanced CT (CECT) thorax revealed asymmetric mid-esophageal wall thickening with lymphadenopathy and metastasis in both hepatic lobes. Esophageal endoscopy revealed a large circumferential ulcero-proliferative mass and umbilicated lesions. A histopathological examination revealed small cells with scant cytoplasm and pleomorphic, hyperchromatic nuclei with prominent nuclear molding, and extensive necrosis. Immunohistochemistry revealed positivity for synaptophysin, chromogranin A, and CD56. Ki-67 index was 53%. These findings suggested poorly differentiated, small cell type, high-grade E-NEC. Chemotherapy with cisplatin (30mg/m2) + irinotecan (60mg/m2) was initiated. However, following two chemotherapy cycles, the patient succumbed.
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Affiliation(s)
- Kamlesh Taori
- Department of Gastroenterology and Hepatology, Jawaharlal Nehru Medical College, Datta Meghe Institute of Medical Sciences, Wardha, IND
| | - Vijendra Kirnake
- Department of Gastroenterology, Jawaharlal Nehru Medical College, Datta Meghe Institute of Medical Sciences, Wardha, IND
| | - Parmeshwar R Junare
- Department of Gastroenterology, Jawaharlal Nehru Medical College, Datta Meghe Institute of Medical Sciences, Wardha, IND
| | - Vishal Padwale
- Department of Gastroenterology, Jawaharlal Nehru Medical College, Datta Meghe Institute of Medical Sciences, Wardha, IND
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4
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Zhong L, Pan Y, Han S, Qi Q, Liao H, Jiang Y, Shen J. The different prognostic factors between metastatic and nonmetastatic disease of esophageal neuroendocrine carcinoma. Indian J Cancer 2023; 60:512-520. [PMID: 38206083 DOI: 10.4103/ijc.ijc_151_20] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/25/2020] [Accepted: 03/01/2021] [Indexed: 01/12/2024]
Abstract
PURPOSE The specific risk factors of metastatic and nonmetastatic esophageal neuroendocrine carcinoma (NEC) are still uncertain. Whether primary site surgery is necessary for all patients with esophageal NEC is unknown. METHODS Patients with esophageal NEC in the Surveillance, Epidemiology, and End Results (SEER) database from 2004 to 2014 were selected. STATA 12 was used to analyze the clinical and pathological features of esophageal NEC. RESULTS In total, 241 patients with esophageal NEC were included. Metastatic patients had shorter overall survival than nonmetastatic patients (6.03 versus 11.90 months, respectively). Prognostic factors varied between metastatic and nonmetastatic esophageal NEC. The location of the primary tumor is a key point for the prognosis of esophageal NEC. For nonmetastatic esophageal NEC, patients with tumors in the upper third of the esophagus had the worst survival, and patients with metastatic esophageal NEC with a primary tumor in the lower part of the esophagus tended to have an increased risk of death. Moreover, age ≥68 years (hazard ratio [HR] = 2.05; 95% confidence interval [CI]: 1.28-3.31; P < 0.01) and large cell carcinoma (HR = 2.79; 95% CI: 1.30-6.00; P < 0.01) were independent risk factors in patients with metastatic esophageal NEC. Primary site resection benefited patients with nonmetastatic esophageal NEC (HR = 0.20; 95% CI: 0.07-0.56; P < 0.01) rather than patients with metastatic esophageal NEC (HR = 0.91; 95% CI: 0.29-2.83; P > 0.05). CONCLUSIONS Our study presented that primary tumor location is an important risk factor for nonmetastatic esophageal NEC patients. Age and pathological type are important risk factors for patients with metastatic esophageal NEC. Nonmetastatic esophageal NEC will benefit from primary tumor resection. Systematic treatment is recommended for metastatic esophageal NEC.
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Affiliation(s)
- Liping Zhong
- Department of Medical Oncology, Huzhou Central Hospital, Affiliated Central Hospital HuZhou University, Zhejiang, China
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5
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Zhou L, Xu G, Chen T, Wang Q, Zhao J, Zhang T, Duan R, Xia Y. Anlotinib plus camrelizumab achieved long-term survival in a patient with metastatic esophageal neuroendocrine carcinoma. Cancer Rep (Hoboken) 2023; 6:e1855. [PMID: 37381647 PMCID: PMC10480405 DOI: 10.1002/cnr2.1855] [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: 04/21/2023] [Revised: 06/06/2023] [Accepted: 06/12/2023] [Indexed: 06/30/2023] Open
Abstract
BACKGROUND Esophageal neuroendocrine carcinoma (NEC) is a rare cancer with an extremely poor prognosis. The average overall survival of patients with metastatic disease is only 1 year. The efficacy of anti-angiogenic agents combined with immune checkpoint inhibitors remains unknown. CASE PRESENTATION A 64-year-old man, initially diagnosed with esophageal NEC, underwent neoadjuvant chemotherapy and esophagectomy. Although the patient remained disease-free for 11 months, eventually the tumor progressed and did not respond to three lines of combined therapy (etoposide plus carboplatin with local radiotherapy, albumin-bound paclitaxel plus durvalumab, and irinotecan plus nedaplatin). The patient then received anlotinib plus camrelizumab, and a dramatic regression was observed (confirmed by positron emission tomography-computed tomography). The patient has been disease-free for over 29 months and has survived for over 4 years since diagnosis. CONCLUSION Combined therapy with anti-angiogenic agents and immune checkpoint inhibitors may be a promising strategy for esophageal NEC, although more evidence is warranted to validate its efficacy.
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Affiliation(s)
- Lingxiao Zhou
- Key Laboratory of Respiratory Disease of Zhejiang Province, Department of Respiratory and Critical Care MedicineSecond Affiliated Hospital of Zhejiang University School of MedicineHangzhouZhejiangChina
- Cancer CenterZhejiang UniversityHangzhouZhejiangChina
| | - Guanxin Xu
- Department of Thoracic SurgerySecond Affiliated Hospital of Zhejiang University School of MedicineHangzhouZhejiangChina
| | - Tianwei Chen
- Key Laboratory of Respiratory Disease of Zhejiang Province, Department of Respiratory and Critical Care MedicineSecond Affiliated Hospital of Zhejiang University School of MedicineHangzhouZhejiangChina
- Cancer CenterZhejiang UniversityHangzhouZhejiangChina
| | - Qiyuan Wang
- Department of RadiologySecond Affiliated Hospital of Zhejiang University School of MedicineHangzhouZhejiangChina
| | - Jing Zhao
- Department of Medical OncologySecond Affiliated Hospital of Zhejiang University School of MedicineHangzhouZhejiangChina
| | - Ting Zhang
- Department of Radiation OncologySecond Affiliated Hospital of Zhejiang University School of MedicineHangzhouZhejiangChina
| | - Rong Duan
- Department of PathologySecond Affiliated Hospital of Zhejiang University School of MedicineHangzhouZhejiangChina
| | - Yang Xia
- Key Laboratory of Respiratory Disease of Zhejiang Province, Department of Respiratory and Critical Care MedicineSecond Affiliated Hospital of Zhejiang University School of MedicineHangzhouZhejiangChina
- Cancer CenterZhejiang UniversityHangzhouZhejiangChina
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Gopakumar H, Jahagirdar V, Koyi J, Dahiya DS, Goyal H, Sharma NR, Perisetti A. Role of Advanced Gastrointestinal Endoscopy in the Comprehensive Management of Neuroendocrine Neoplasms. Cancers (Basel) 2023; 15:4175. [PMID: 37627203 PMCID: PMC10453187 DOI: 10.3390/cancers15164175] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/17/2023] [Revised: 08/15/2023] [Accepted: 08/15/2023] [Indexed: 08/27/2023] Open
Abstract
Neuroendocrine neoplasms (NENs), also called neuroendocrine tumors (NETs), are relatively uncommon, heterogenous tumors primarily originating in the gastrointestinal tract. With the improvement in technology and increasing use of cross-sectional imaging and endoscopy, they are being discovered with increasing frequency. Although traditionally considered indolent tumors with good prognoses, some NENs exhibit aggressive behavior. Timely diagnosis, risk stratification, and management can often be a challenge. In general, small NENs without local invasion or lymphovascular involvement can often be managed using minimally invasive advanced endoscopic techniques, while larger lesions and those with evidence of lymphovascular invasion require surgery, systemic therapy, or a combination thereof. Ideal management requires a comprehensive and accurate understanding of the stage and grade of the tumor. With the recent advancements, a therapeutic advanced endoscopist can play a pivotal role in diagnosing, staging, and managing this rare condition. High-definition white light imaging and digital image enhancing technologies like narrow band imaging (NBI) in the newer endoscopes have improved the diagnostic accuracy of traditional endoscopy. The refinement of endoscopic ultrasound (EUS) over the past decade has revolutionized the role of endoscopy in diagnosing and managing various pathologies, including NENs. In addition to EUS-directed diagnostic biopsies, it also offers the ability to precisely assess the depth of invasion and lymphovascular involvement and thus stage NENs accurately. EUS-directed locoregional ablative therapies are increasingly recognized as highly effective, minimally invasive treatment modalities for NENs, particularly pancreatic NENs. Advanced endoscopic resection techniques like endoscopic submucosal dissection (ESD), endoscopic submucosal resection (EMR), and endoscopic full-thickness resection (EFTR) have been increasingly used over the past decade with excellent results in achieving curative resection of various early-stage gastrointestinal luminal lesions including NENs. In this article, we aim to delineate NENs of the different segments of the gastrointestinal (GI) tract (esophagus, gastric, pancreatic, and small and large intestine) and their management with emphasis on the endoscopic management of these tumors.
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Affiliation(s)
- Harishankar Gopakumar
- Department of Gastroenterology and Hepatology, University of Illinois College of Medicine at Peoria, Peoria, IL 61605, USA;
| | - Vinay Jahagirdar
- Department of Internal Medicine, University of Missouri-Kansas City, Kansas City, MO 64110, USA; (V.J.); (J.K.)
| | - Jagadish Koyi
- Department of Internal Medicine, University of Missouri-Kansas City, Kansas City, MO 64110, USA; (V.J.); (J.K.)
| | - Dushyant Singh Dahiya
- Division of Gastroenterology, Hepatology & Motility, The University of Kansas School of Medicine, Kansas City, KS 66160, USA;
| | - Hemant Goyal
- Department of Surgery, Center for Interventional Gastroenterology at UT (iGUT), The University of Texas Health Science Center, Houston, TX 77054, USA;
| | - Neil R. Sharma
- Advanced Interventional Endoscopy & Endoscopic Oncology (IOSE) Division, GI Oncology Tumor Site Team, Parkview Cancer Institute, 11104 Parkview Circle, Suite 310, Fort Wayne, IN 46845, USA;
| | - Abhilash Perisetti
- Division of Gastroenterology and Hepatology, Kansas City Veteran Affairs, Kansas City, MO 64128, USA
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Patel P, Zaher EA, Sqour H. High-Grade Pure Esophageal Neuroendocrine Carcinoma Arising From Barrett's Mucosa: A Rare Phenomenon. Cureus 2023; 15:e40644. [PMID: 37476144 PMCID: PMC10355687 DOI: 10.7759/cureus.40644] [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: 06/18/2023] [Indexed: 07/22/2023] Open
Abstract
Esophageal neuroendocrine carcinoma (E-NEC) is a very rare neuroendocrine tumor. There are only a few case reports where pure esophageal NEC is found to be arising from Barett's mucosa. Here we present a case of high-grade pure E-NEC arising from Barrett's esophagus, which was metastasized to the liver at the time of diagnosis.
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Affiliation(s)
- Parth Patel
- Internal Medicine, Ascension Saint Joseph Hospital, Chicago, USA
| | - Eli A Zaher
- Internal Medicine, Ascension Saint Joseph Hospital, Chicago, USA
| | - Hasan Sqour
- Internal Medicine, Ascension Saint Joseph Hospital, Chicago, USA
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8
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Awada H, Hajj Ali A, Bakhshwin A, Daw H. High-grade large cell neuroendocrine carcinoma of the esophagus: a case report and review of the literature. J Med Case Rep 2023; 17:144. [PMID: 37024958 PMCID: PMC10080843 DOI: 10.1186/s13256-023-03879-0] [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: 07/13/2022] [Accepted: 03/07/2023] [Indexed: 04/08/2023] Open
Abstract
BACKGROUND Neuroendocrine carcinomas are extremely rare in the esophagus as they represent less than 0.04% of all neuroendocrine tumors. To date, only 14 cases of poorly differentiated, high-grade esophageal NEC have been described in the literature. The majority of these patients presented with typical dysphagia symptoms. Due to its rarity, no standardized guidelines have been proposed to treat esophageal neuroendocrine carcinoma, although general recommendations suggest surgery with adjuvant chemoradiotherapy as the treatment of choice. CASE PRESENTATION A 67-year-old previously healthy White male presented with a year-long intermittent nonspecific retrosternal discomfort, with the absence of any other symptoms. Esophagogastroduodenoscopy revealed an ulcerative mass in his lower esophagus, with concern of malignancy. Endoscopic ultrasound-guided biopsy revealed poorly differentiated neuroendocrine carcinoma of the esophagus with metastasis to a diaphragmatic lymph node. He was treated with neoadjuvant chemoradiation followed by surgery, and he has been in remission for over 5 years. CONCLUSION Here, we review the literature and report a unique case of a patient with a vague presentation of esophageal neuroendocrine carcinoma as he enters his sixth year of survival following neoadjuvant chemoradiotherapy.
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Affiliation(s)
- Hussein Awada
- Department of Translational Hematology and Oncology Research, Taussig Cancer Institute, Cleveland Clinic, Cleveland, OH, 44195, USA.
| | - Adel Hajj Ali
- Heart, Vascular & Thoracic Institute, Cleveland Clinic, Cleveland, OH, 44195, USA
| | - Ahmed Bakhshwin
- Pathology Department, Robert J. Tomsich Pathology & Laboratory Medicine Institute, Cleveland Clinic, Cleveland, OH, 44195, USA
| | - Hamed Daw
- Hematology and Oncology, Cleveland Clinic Fairview Hospital, Cleveland, OH, 44111, USA
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9
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Zhang L, Yu B, Liu Z, Wei J, Pan J, Jiang C, Li Z. Analysis of the Clinicopathological Characteristics, Prognosis, and Lymphocyte Infiltration of Esophageal Neuroendocrine Neoplasms: A Surgery-Based Cohort and Propensity-Score Matching Study. Cancers (Basel) 2023; 15:cancers15061732. [PMID: 36980618 PMCID: PMC10046526 DOI: 10.3390/cancers15061732] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/17/2022] [Revised: 01/31/2023] [Accepted: 02/22/2023] [Indexed: 03/14/2023] Open
Abstract
Background: Esophageal neuroendocrine neoplasms (E-NENs) are a rare and poorly reported subtype of esophageal carcinoma. We analyzed the differences in clinicopathological features, prognosis, and tumor-infiltrating lymphocytes (TILs) between E-NENs and esophageal squamous cell carcinoma (ESCC). Methods: A total of 3620 patients who underwent esophagectomy were enrolled retrospectively. The study cohort was divided into two groups (E-NENs and ESCC) through propensity-score matching, and the prognosis and TILs were compared between the two groups. The TILs were assessed using tumor specimens (including six cases of ESCC, six cases of neuroendocrine carcinomas [NECs], and six cases of mixed neuroendocrine–non-neuroendocrine neoplasms [MiNENs]). Results: E-NENs accounted for 3.0% (107/3620) of cases, among which there were just 3 neuroendocrine tumor cases, 51 NEC cases, and 53 MiNENs cases. After matching, esophageal neuroendocrine carcinomas (E-NECs) showed both poorer 5-year overall survival (OS; 35.4% vs. 54.8%, p = 0.0019) and recurrence-free survival (RFS; 29.3% vs. 48.9%, p < 0.001) compared with ESCC. However, the differences were not prominent in the subgroup with stage I. No significant survival benefit was observed for E-NECs with multimodal therapy. Multivariate analysis demonstrated that E-NECs are an independent risk factor for OS and RFS. In the exploratory analysis, E-NECs were associated with less infiltration of immune cells compared with ESCC. Conclusion: E-NECs are significantly associated with a poorer prognosis than ESCC except for early-stage disease. The fewer TILs within the tumor microenvironment of E-NECs compared with ESCC results in weaker anti-tumor immunity and may lead to a poorer prognosis.
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Affiliation(s)
- Long Zhang
- Department of Thoracic Surgery, Section of Esophageal Surgery, Shanghai Chest Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai 200030, China
| | - Boyao Yu
- Department of Thoracic Surgery, Section of Esophageal Surgery, Shanghai Chest Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai 200030, China
| | - Zhichao Liu
- Department of Thoracic Surgery, Section of Esophageal Surgery, Shanghai Chest Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai 200030, China
| | - Jinzhi Wei
- Department of Pathology, Shanghai Chest Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai 200030, China
| | - Jie Pan
- Department of Thoracic Surgery, Section of Esophageal Surgery, Shanghai Chest Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai 200030, China
| | - Chao Jiang
- Department of Thoracic Surgery, Section of Esophageal Surgery, Shanghai Chest Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai 200030, China
| | - Zhigang Li
- Department of Thoracic Surgery, Section of Esophageal Surgery, Shanghai Chest Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai 200030, China
- Correspondence:
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Vest M, Shah D, Nassar M, Niknam N. Neuroendocrine Carcinoma of the Esophagus With Liver Metastasis: A Case Report. Cureus 2022; 14:e28842. [PMID: 36225482 PMCID: PMC9536476 DOI: 10.7759/cureus.28842] [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: 09/06/2022] [Indexed: 11/05/2022] Open
Abstract
Neuroendocrine carcinoma (NEC) of the esophagus is a rare and aggressive malignancy. It is challenging to manage NEC due to its rarity. NEC may be asymptomatic or present with various symptoms such as dysphagia, abdominal discomfort, weight loss, melena, hot flushes, or diarrhea. We present the case of a 55-year-old male with a large cell neuroendocrine carcinoma of the esophagus. His aggressive and rapid progression of neuroendocrine carcinoma of the esophagus resulted in a poor clinical outcome.
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Affiliation(s)
- Mallorie Vest
- Internal Medicine, Icahn School of Medicine at Mount Sinai/NYC Health+Hospitals Queens, New York City, USA
| | - Deesha Shah
- Internal Medicine, Icahn School of Medicine at Mount Sinai/NYC Health+Hospitals Queens, New York City, USA
| | - Mahmoud Nassar
- Internal Medicine, Icahn School of Medicine at Mount Sinai/NYC Health+Hospitals Queens, New York City, USA
| | - Negar Niknam
- Gastroenterology, Icahn School of Medicine at Mount Sinai/NYC Health+Hospitals Queens, New York City, USA
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11
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Chen C, Hu H, Zheng Z, Yang Y, Chen W, Qiao X, Li P, Zhang S. Clinical characteristics, prognostic factors, and survival trends in esophageal neuroendocrine carcinomas: A population-based study. Cancer Med 2022; 11:4935-4945. [PMID: 35596661 PMCID: PMC9761068 DOI: 10.1002/cam4.4829] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/08/2022] [Revised: 04/14/2022] [Accepted: 05/01/2022] [Indexed: 02/03/2023] Open
Abstract
BACKGROUND Esophageal neuroendocrine carcinoma (ENEC) is an extremely rare type of malignancy. Clinical data of ENEC are limited to case reports and case series. More information is needed on its clinical feature, management, and prognosis. METHODS This study collected information of ENEC patients diagnosed pathologically from 2010 to 2018. Data including demographic information, clinical features, and survival trends were obtained from the Surveillance, Epidemiology, and End Results (SEER) database. Statistical analyses were performed with STATA/SE 15.1, SPSS 25.0, and GraphPad Prism 8. RESULTS A total of 283 ENEC patients were included in this study. The small-cell and large-cell subtypes of ENEC possess similar clinical features. The lower third of the esophagus (58%) was the most common location of ENEC. At the time of diagnosis, most ENEC patients were AJCC 7th stage IV (48.1%). Metastasis occurred in more than half of the ENEC patients (53.4%), and the most common metastatic site was the liver (37.1%). Compared with poorly differentiated esophageal squamous cell carcinoma (ESCC), another aggressive malignancy of the esophagus sometimes confused with ENEC because of similar histological features, our study showed differences in tumor location and metastatic rate, but similar poor survival rates. Multivariate survival analysis showed that ENEC located at the middle third of esophagus (p = 0.013), "Brain metastasis" (p = 0.019), and "Liver metastasis" (p < 0.001) were independent predictors of worse outcomes. "Surgery" (p = 0.003), and "Chemotherapy" (p < 0.001) were associated with better survival. CONCLUSION A significant proportion of patients with newly diagnosed ENEC presented with metastatic disease. Predictors of poor survival included tumor location, brain metastasis, and liver metastasis. ENEC and poorly differentiated ESCC share certain histological features, but differ in tumor location and metastatic rate. Yet, no standard treatment strategy has been established, but surgery and chemotherapy were related to better outcomes.
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Affiliation(s)
- Chuyan Chen
- Department of Gastroenterology, Beijing Friendship Hospital, Beijing Key Laboratory for Precancerous Lesion of Digestive DiseaseNational Clinical Research Center for Digestive Disease, Beijing Digestive Disease Center, Capital Medical UniversityBeijingChina
| | - Haiyi Hu
- Department of Gastroenterology, Beijing Friendship Hospital, Beijing Key Laboratory for Precancerous Lesion of Digestive DiseaseNational Clinical Research Center for Digestive Disease, Beijing Digestive Disease Center, Capital Medical UniversityBeijingChina
| | - Zhibo Zheng
- Department of International Medical ServicesPeking Union Medical College Hospital, Chinese Academy of Medical SciencesBeijingChina,Department of Thoracic SurgeryPeking Union Medical College Hospital, Chinese Academy of Medical SciencesBeijingChina
| | - Yi Yang
- Department of Gastroenterology, Beijing Friendship Hospital, Beijing Key Laboratory for Precancerous Lesion of Digestive DiseaseNational Clinical Research Center for Digestive Disease, Beijing Digestive Disease Center, Capital Medical UniversityBeijingChina
| | - Wei Chen
- Department of Gastroenterology, Beijing Friendship Hospital, Beijing Key Laboratory for Precancerous Lesion of Digestive DiseaseNational Clinical Research Center for Digestive Disease, Beijing Digestive Disease Center, Capital Medical UniversityBeijingChina
| | - Xinwei Qiao
- Department of Gastroenterology, Beijing Friendship Hospital, Beijing Key Laboratory for Precancerous Lesion of Digestive DiseaseNational Clinical Research Center for Digestive Disease, Beijing Digestive Disease Center, Capital Medical UniversityBeijingChina
| | - Peng Li
- Department of Gastroenterology, Beijing Friendship Hospital, Beijing Key Laboratory for Precancerous Lesion of Digestive DiseaseNational Clinical Research Center for Digestive Disease, Beijing Digestive Disease Center, Capital Medical UniversityBeijingChina
| | - Shutian Zhang
- Department of Gastroenterology, Beijing Friendship Hospital, Beijing Key Laboratory for Precancerous Lesion of Digestive DiseaseNational Clinical Research Center for Digestive Disease, Beijing Digestive Disease Center, Capital Medical UniversityBeijingChina
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Fang JM, Li J, Shi J. An update on the diagnosis of gastroenteropancreatic neuroendocrine neoplasms. World J Gastroenterol 2022; 28:1009-1023. [PMID: 35431496 PMCID: PMC8968521 DOI: 10.3748/wjg.v28.i10.1009] [Citation(s) in RCA: 26] [Impact Index Per Article: 8.7] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/17/2021] [Revised: 11/26/2021] [Accepted: 02/10/2022] [Indexed: 02/06/2023] Open
Abstract
Gastroenteropancreatic neuroendocrine neoplasms (GEP-NENs) arise from neuroendocrine cells found throughout the gastrointestinal tract and islet cells of the pancreas. The incidence and prevalence of GEP-NENs have been increasing each year due to higher awareness, improved diagnostic modalities, and increased incidental detection on cross-sectional imaging and endoscopy for cancer screening and other conditions and symptoms. GEP-NENs are a heterogeneous group of tumors and have a wide range in clinical presentation, histopathologic features, and molecular biology. Clinical presentation most commonly depends on whether the GEP-NEN secretes an active hormone. The World Health Organization recently updated the classification of GEP-NENs to introduce a distinction between high-grade neuroendocrine tumors and neuroendocrine carcinomas, which can be identified using histology and molecular studies and are more aggressive with a worse prognosis compared to high-grade neuroendocrine tumors. As our understanding of the biology of GEP-NENs has grown, new and improved diagnostic modalities can be developed and optimized. Here, we discuss clinical features and updates in diagnosis, including histopathological analysis, biomarkers, molecular techniques, and radiology of GEP-NENs. We review established diagnostic tests and discuss promising novel diagnostic tests that are currently in development or require further investigation and validation prior to broad utilization in patient care.
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Affiliation(s)
- Jiayun M Fang
- Department of Pathology & Clinical Labs, University of Michigan, Ann Arbor, MI 48109, United States
| | - Jay Li
- Medical Scientist Training Program, University of Michigan, Ann Arbor, MI 48109, United States
| | - Jiaqi Shi
- Department of Pathology & Clinical Labs, University of Michigan, Ann Arbor, MI 48109, United States
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13
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Galanis I, Simou M, Floros G. Large-Cell Esophageal Neuroendocrine Carcinoma: Report of a Rare Case. Cureus 2022; 14:e22041. [PMID: 35340509 PMCID: PMC8913815 DOI: 10.7759/cureus.22041] [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: 02/08/2022] [Indexed: 12/02/2022] Open
Abstract
Neuroendocrine tumors (NETs) are neoplasms with neuroendocrine characteristics such as secretion of neuropeptides, large secretory vesicles, and a lack of neural structures. Neuroendocrine carcinoma (NEC) of the esophagus is a very rare malignancy. We present the case of a 58-year-old male with a pure large cell NEC of the esophagus.
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14
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Liu S, Ge X, Gao Z, Zhou Q, Shi Y, Jiang W, Yang M, Sun X. Clinicopathological analysis of 67 cases of esophageal neuroendocrine carcinoma and the effect of postoperative adjuvant therapy on prognosis. Medicine (Baltimore) 2021; 100:e27302. [PMID: 34713821 PMCID: PMC8556034 DOI: 10.1097/md.0000000000027302] [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: 02/23/2020] [Accepted: 09/02/2021] [Indexed: 01/05/2023] Open
Abstract
The clinicopathological properties of esophageal neuroendocrine carcinoma (ENEC) and its optimal therapy have not been widely studied, as the disease is not common. Consequently, we conducted a retrospective study to analyze the clinical features as well as the prognosis of patients with surgically resected ENEC.The clinicopathological data of patients with ENEC who underwent esophagostomy with regional lymphadenectomy at Jiangsu Province People's Hospital and Jiangsu Provincial Tumor Hospital starting January 2008 until December 2014 were collected.Ninety-two cases of ENEC were part of this study. However, only 67 patients were analyzed and followed up. A univariate model for the Cox proportional hazards revealed that prognosis was associated with postoperative adjuvant therapy, age, and lymph node metastasis (P < .05); a multivariate Cox proportional hazards model showed that postoperative adjuvant therapy was a significant independent prognostic factor. Postoperative adjuvant therapy directly affected overall survival, with a significant disparity noted between the groups (P = .022). In this study, patients who received adjuvant therapy had an average time of survival of 39 months (interquartile range: 27.068-50.932 months), while those who did not receive adjuvant therapy had an average survival time of 13 months (interquartile range: 10.129-15.871 months). The survival time was longer in the treated group than in the untreated group (hazard ratio = 0.47; 95% confidence interval: 0.23-0.94; P = .034).ENEC is a heterogeneous tumor with a very poor prognosis. Combining surgery with adjuvant and/or chemotherapy significantly prolongs the survival of patients, and the optimal treatment for ENEC should be determined with future prospective studies.
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Affiliation(s)
- Shenxiang Liu
- Department of Radiotherapy, The First Affiliated Hospital of Nanjing Medical University, Jiangsu, China
- Department of Oncology, The Affiliated Hospital of Yangzhou University, Yangzhou University, Jiangsu, China
| | - Xiaolin Ge
- Department of Radiotherapy, The First Affiliated Hospital of Nanjing Medical University, Jiangsu, China
| | - Zhenzhen Gao
- Department of Oncology, The Second Affiliated Hospital of Jiaxing University, Zhe Jiang, China
| | - Qing Zhou
- Department of Oncology, Jiangsu Provincial Tumor Hospital, Jiangsu, China
| | - Yu Shi
- Department of Radiotherapy, The First Affiliated Hospital of Nanjing Medical University, Jiangsu, China
| | - Wangrong Jiang
- Department of Radiotherapy, People's Liberation Army (PLA) 81 Hospital, Jiangsu, China
| | - Min Yang
- Department of Radiotherapy, The First Affiliated Hospital of Nanjing Medical University, Jiangsu, China
- Jiangsu Institute of Nuclear Medicine, Jiangsu, China
| | - Xinchen Sun
- Department of Radiotherapy, The First Affiliated Hospital of Nanjing Medical University, Jiangsu, China
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15
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Sohda M, Saeki H, Kuwano H, Miyazaki T, Yokobori T, Sano A, Sakai M, Kakeji Y, Toh Y, Doki Y, Matsubara H. Diagnostic Immunostaining and Tumor Markers Predict the Prognosis of Esophageal Neuroendocrine Cell Carcinoma Patients. Ann Surg Oncol 2021; 28:7983-7989. [PMID: 33843025 DOI: 10.1245/s10434-021-09872-5] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/21/2021] [Accepted: 02/26/2021] [Indexed: 01/16/2023]
Abstract
BACKGROUND Esophageal neuroendocrine carcinoma (ENEC) has a poor prognosis, and predicting the prognosis by examining various markers may contribute to the determination of treatment strategies. Therefore, a multiple-institution retrospective study was performed to identify biomarkers using diagnostic immunohistochemistry and serum tumor markers that predict the prognosis of patients with ENEC. METHODS The results of immunohistochemical examination and serum tumor markers were extracted from the data of 141 ENEC patients at 39 institutions certified by the Japan Esophageal Society. The study then examined correlations between these data and prognosis or treatment effects. RESULTS The ENEC patients with positively for all expression of synaptophysin (Syn), chromogranin A (CgA), and CD56 had a significantly worse prognosis than the patients with other expression patterns. Additionally, surgery and chemoradiotherapy were significantly more effective treatments than chemotherapy for the patients who were not positive for all expressions of Syn, CgA, and CD56. In terms of serum tumor markers, the patients with a high neuron-specific enolase (NSE) value had a significantly worse prognosis than the patients with a normal NSE value, and complete response (CR) cases treated with chemotherapy were significantly fewer in the high-NSE group. The results of multivariate analysis demonstrated that high NSE levels were an independent poor prognostic factor for esophageal endocrine cell carcinoma. CONCLUSION This study showed that positivity for all expressions of Syn, CgA, and CD56, and a high NSE value were significantly worse prognostic factors for ENEC patients than other expression patterns and may be important prognostic biomarkers of ENEC.
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Affiliation(s)
- Makoto Sohda
- Department of General Surgical Science, Graduate School of Medicine, Gunma University, Maebashi, Gunma, Japan.
| | - Hiroshi Saeki
- Department of General Surgical Science, Graduate School of Medicine, Gunma University, Maebashi, Gunma, Japan
| | - Hiroyuki Kuwano
- Department of General Surgical Science, Graduate School of Medicine, Gunma University, Maebashi, Gunma, Japan
| | - Tatsuya Miyazaki
- Department of General Surgical Science, Graduate School of Medicine, Gunma University, Maebashi, Gunma, Japan
| | - Takehiko Yokobori
- Department of General Surgical Science, Graduate School of Medicine, Gunma University, Maebashi, Gunma, Japan
| | - Akihiko Sano
- Department of General Surgical Science, Graduate School of Medicine, Gunma University, Maebashi, Gunma, Japan
| | - Makoto Sakai
- Department of General Surgical Science, Graduate School of Medicine, Gunma University, Maebashi, Gunma, Japan
| | - Yoshihiro Kakeji
- Division of Gastrointestinal Surgery, Department of Surgery, Kobe University Graduate School of Medicine, Kobe, Japan
| | - Yasushi Toh
- Department of Gastroenterological Surgery, National Hospital Organization Kyushu Cancer Center, Fukuoka, Japan
| | - Yuichiro Doki
- Department of Gastroenterological Surgery, Graduate School of Medicine, Osaka University, Suita, Japan
| | - Hisahiro Matsubara
- Department of Frontier Surgery, Graduate School of Medicine, Chiba University, Chiba, Japan
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16
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Nationwide survey of neuroendocrine carcinoma of the esophagus: a multicenter study conducted among institutions accredited by the Japan Esophageal Society. J Gastroenterol 2021; 56:350-359. [PMID: 33582864 DOI: 10.1007/s00535-020-01756-x] [Citation(s) in RCA: 4] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/03/2020] [Accepted: 11/30/2020] [Indexed: 02/04/2023]
Abstract
BACKGROUND Esophageal neuroendocrine carcinoma (ENEC) is an extremely rare type of tumor characterized by a high malignant potential, rapid growth, and poor prognosis. Because the standard therapy for ENEC has been poorly defined, we herein aimed to attain a clear view of the current state of treatment for ENEC by performing a large-scale, multicenter study. METHODS We conducted a questionnaire-based, retrospective clinical review of 142 patients with ENEC treated at 37 hospitals accredited by the Japan Esophageal Society from January 2010 to December 2015. RESULTS The most frequent main treatment performed was an operation, followed by chemoradiotherapy, chemotherapy, and endoscopic submucosal dissection. Among the 67 patients who underwent surgery, 28 (19.7%) were not accurately diagnosed with NEC or a combined NEC tumor by pretreatment biopsy. The 5-year overall survival rate of all patients was 33.2%. Among patients with Stages III and IV disease, the prognosis was significantly better in patients who underwent chemoradiotherapy than in patients who underwent other treatments. Among patients with Stages I and II disease, however, there was no difference in survival rates between those who underwent surgery and chemoradiotherapy. CONCLUSIONS This nationwide survey is a valuable report of the current status of treatment of ENEC in a limited number of cases experienced at each participating institution. The survival benefit obtained from surgery was considered to be limited, especially for Stages III and IV ENEC. Accurate pretreatment histological diagnosis is essential to determine the most appropriate treatment strategy for patients with ENEC.
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17
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Cai MY, Cardoso MF, Zhu Y, Zhong YS, Zhou PH. An esophageal submucosal tumor treated with submucosal tunneling endoscopic resection: an unexpected result. Gastroenterol Rep (Oxf) 2020; 9:269-271. [PMID: 34316377 PMCID: PMC8309675 DOI: 10.1093/gastro/goaa073] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/15/2020] [Accepted: 03/30/2020] [Indexed: 11/24/2022] Open
Affiliation(s)
- Ming-Yan Cai
- Endoscopy Center, Zhongshan Hospital, Fudan University, Shanghai, P.R. China
| | | | - Yan Zhu
- Endoscopy Center, Zhongshan Hospital, Fudan University, Shanghai, P.R. China
| | - Yun-Shi Zhong
- Endoscopy Center, Zhongshan Hospital, Fudan University, Shanghai, P.R. China
| | - Ping-Hong Zhou
- Endoscopy Center, Zhongshan Hospital, Fudan University, Shanghai, P.R. China
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18
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Zhou Y, Hou P, Zha KJ, Wang F, Zhou K, He W, Gao JB. Prognostic value of pretreatment contrast-enhanced computed tomography in esophageal neuroendocrine carcinoma: A multi-center follow-up study. World J Gastroenterol 2020; 26:4680-4693. [PMID: 32884225 PMCID: PMC7445868 DOI: 10.3748/wjg.v26.i31.4680] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/05/2020] [Revised: 05/22/2020] [Accepted: 07/30/2020] [Indexed: 02/06/2023] Open
Abstract
BACKGROUND The rare incidence of esophageal neuroendocrine carcinoma (NEC) and limited treatment experience result in insufficient clinical observations and unsuitable guidelines for its management.
AIM To investigate the prognostic value of pretreatment contrast-enhanced computed tomography (CT) characteristics in patients with esophageal NEC.
METHODS Seventy-seven esophageal NEC patients who received contrast-enhanced CT at two hospitals were enrolled in this study from June 2014 to December 2019. The clinical features and image characteristics were recorded accordingly. Univariate survival analysis was performed using the Kaplan-Meier method and log-rank test, and multivariate analysis was carried out with a Cox proportional hazards model.
RESULTS The multivariate analysis performed using the Cox proportional hazards model showed that N stage, adjuvant chemotherapy, and degree of enhancement were independent prognostic factors for overall survival (OS). Meanwhile, adjuvant chemotherapy was an independent prognostic factor for progression-free survival (PFS). The hazard ratios (HRs) of N stage, adjuvant chemotherapy, and degree of enhancement (mild vs moderate/marked) for OS were 0.426 (P = 0.024), 3.862 (P = 0.006), and 2.169/0.809 (P = 0.037), respectively. The HR of adjuvant chemotherapy for PFS was 6.432 (P < 0.001). Adjuvant chemotherapy was significantly associated with degree of enhancement (P = 0.018).
CONCLUSION Adjuvant chemotherapy is an independent prognostic factor for OS and PFS. Additionally, N stage and degree of enhancement are prognostic factors for OS in patients with esophageal NEC.
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Affiliation(s)
- Yue Zhou
- Department of Radiology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou 450052, Henan Province, China
| | - Ping Hou
- Department of Radiology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou 450052, Henan Province, China
| | - Kai-Ji Zha
- Department of Radiology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou 450052, Henan Province, China
| | - Feng Wang
- Department of Oncology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou 450052, Henan Province, China
| | - Kun Zhou
- Department of Thoracic Surgery, The First Affiliated Hospital of Zhengzhou University, Zhengzhou 450052, Henan Province, China
| | - Wei He
- Department of Oncology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou 450052, Henan Province, China
| | - Jian-Bo Gao
- Department of Radiology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou 450052, Henan Province, China
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Businello G, Dal Pozzo CA, Sbaraglia M, Mastracci L, Milione M, Saragoni L, Grillo F, Parente P, Remo A, Bellan E, Cappellesso R, Pennelli G, Michelotto M, Fassan M. Histopathological landscape of rare oesophageal neoplasms. World J Gastroenterol 2020; 26:3865-3888. [PMID: 32774063 PMCID: PMC7385561 DOI: 10.3748/wjg.v26.i27.3865] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/25/2020] [Revised: 06/19/2020] [Accepted: 07/04/2020] [Indexed: 02/06/2023] Open
Abstract
The landscape of neoplastic pathology of the oesophagus is dominated by malignancies of epithelial origin, in particular by oesophageal adenocarcinoma and oesophageal squamous cell carcinoma. However, several other histopathological variants can be distinguished, some associated with peculiar histopathological profiles and prognostic behaviours and frequently underrecognized in clinical practice. The aim of this review is to provide a comprehensive characterization of the main morphological and clinical features of these rare variants of oesophageal neoplastic lesions.
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Affiliation(s)
- Gianluca Businello
- Department of Medicine (DIMED), Surgical Pathology and Cytopathology Unit, University of Padua, Padua 35121, Italy
| | - Carlo Alberto Dal Pozzo
- Department of Medicine (DIMED), Surgical Pathology and Cytopathology Unit, University of Padua, Padua 35121, Italy
| | - Marta Sbaraglia
- Department of Medicine (DIMED), Surgical Pathology and Cytopathology Unit, University of Padua, Padua 35121, Italy
| | - Luca Mastracci
- Department of Surgical and Diagnostic Sciences (DISC), Pathology Unit, University of Genova, Genova 16123, Italy
| | - Massimo Milione
- Department of Pathology and Laboratory Medicine, First Pathology Division, Fondazione IRCCS - Istituto Nazionale dei Tumori, Milan 20133, Italy
| | - Luca Saragoni
- Pathology Unit, Morgagni-Pierantoni Hospital, Forlì 47121, Italy
| | - Federica Grillo
- Department of Surgical and Diagnostic Sciences (DISC), Pathology Unit, University of Genova, Genova 16123, Italy
| | - Paola Parente
- Pathology Unit, Fondazione IRCCS Casa Sollievo della Sofferenza, San Giovanni Rotondo 71013, Italy
| | - Andrea Remo
- Department of Pathology, Ospedale Mater Salutis di Legnago, Legnago 37045, Italy
| | - Elena Bellan
- Department of Medicine (DIMED), Surgical Pathology and Cytopathology Unit, University of Padua, Padua 35121, Italy
| | - Rocco Cappellesso
- Department of Medicine (DIMED), Surgical Pathology and Cytopathology Unit, University of Padua, Padua 35121, Italy
| | - Gianmaria Pennelli
- Department of Medicine (DIMED), Surgical Pathology and Cytopathology Unit, University of Padua, Padua 35121, Italy
| | - Mauro Michelotto
- Department of Medicine (DIMED), Surgical Pathology and Cytopathology Unit, University of Padua, Padua 35121, Italy
| | - Matteo Fassan
- Department of Medicine (DIMED), Surgical Pathology and Cytopathology Unit, University of Padua, Padua 35121, Italy
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20
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Wu IC, Chu YY, Wang YK, Tsai CL, Lin JC, Kuo CH, Shih HY, Chung CS, Hu ML, Sun WC, Wang JP, Wang HP. Clinicopathological features and outcome of esophageal neuroendocrine tumor: A retrospective multicenter survey by the digestive endoscopy society of Taiwan. J Formos Med Assoc 2020; 120:508-514. [PMID: 32600867 DOI: 10.1016/j.jfma.2020.06.024] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/06/2020] [Revised: 05/15/2020] [Accepted: 06/21/2020] [Indexed: 12/22/2022] Open
Abstract
BACKGROUND & AIMS Esophageal neuroendocrine tumors (NET) are very rare and mostly carcinomic, carrying poor prognosis. There is still no guideline or consensus on the treatment for esophageal NET. METHODS Patients with histologically-proven esophageal neuroendocrine tumor were recruited from 9 hospitals in Taiwan between 2002 and 2017. Clinical, laboratory, radiological, endoscopic, pathological data, treatment strategies, follow-up periods, and survivals were collected retrospectively. RESULTS In total, 39 esophageal NET were analyzed and 38 were neuroendocrine carcinoma (NEC). Sixteen (41%) patients had mixed components with either adenocarcinoma (N = 9, 23%) or squamous-cell carcinoma (SCC) (N = 7, 18%). 64.1% of the patients experienced dysphagia and ulcerative mass was the most comment endoscopic finding. There was a higher proportion of drinkers (54.1%), betel chewers (21.6%) and smokers (64.9%) among the patients than in the general population in Taiwan. Five patients (12.8%) had been diagnosed with other cancers. Definite chemoradiotherapy (N = 14, 35.9%) and surgery (N = 7, 17.9%) were the major treatment. Patients with Ki-67% above the median level (50%) in the tumors tended to have worse survival (P = 0.06). However, presence of mixed component was not a significant survival predictor in our study (P = 0.56). CONCLUSION Mixed component of an esophageal NET is commonly observed. Staged workup and the principle of treatment can follow that for the common cancer type of esophagus. The risk factors and behaviors of esophageal NEC in Taiwan seem to be similar to that of esophageal SCC.
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Affiliation(s)
- I-Chen Wu
- Division of Gastroenterology, Department of Internal Medicine, Kaohsiung Medical University Hospital, Kaohsiung, Taiwan; Department of Medicine, Faculty of Medicine, College of Medicine, Kaohsiung Medical University, Kaohsiung, Taiwan
| | - Yin-Yi Chu
- Department of Gastroenterology and Hepatology, Linkou Chang Gung Memorial Hospital and Chang Gung University, Taoyuan, Taiwan; Department of Gastroenterology and Hepatology, New Taipei Municipal TuCheng Hospital and Chang Gung University, New Taipei City, Taiwan
| | - Yao-Kuang Wang
- Division of Gastroenterology, Department of Internal Medicine, Kaohsiung Medical University Hospital, Kaohsiung, Taiwan; Department of Medicine, Faculty of Medicine, College of Medicine, Kaohsiung Medical University, Kaohsiung, Taiwan
| | - Cho-Lun Tsai
- Department of Internal Medicine, Ditmanson Medical Foundation Chia-Yi Christian Hospital, Chia-Yi, Taiwan
| | - Jung-Chun Lin
- Division of Gastroenterology, Department of Internal Medicine, Tri-Service General Hospital, National Defense Medical Center, Taipei, Taiwan
| | - Chao-Hung Kuo
- Division of Gastroenterology, Department of Internal Medicine, Kaohsiung Medical University Hospital, Kaohsiung, Taiwan; Department of Medicine, Faculty of Medicine, College of Medicine, Kaohsiung Medical University, Kaohsiung, Taiwan; Department of Internal Medicine, Kaohsiung Municipal Hsiao-Kang Hospital, Kaohsiung, Taiwan
| | - Hsiang-Yao Shih
- Division of Gastroenterology, Department of Internal Medicine, Kaohsiung Medical University Hospital, Kaohsiung, Taiwan
| | - Chen-Shuan Chung
- Department of Internal Medicine, Far Eastern Memorial Hospital, New Taipei City, Taiwan; Department of Internal Medicine, National Taiwan University Hospital, National Taiwan University, College of Medicine, Taipei, Taiwan
| | - Ming-Luen Hu
- Division of Hepato-Gastroenterology, Department of Internal Medicine, Kaohsiung Chang Gung Memorial Hospital, Kaohsiung, Taiwan
| | - Wei-Chih Sun
- Division of Gastroenterology and Hepatology, Department of Internal Medicine, Kaohsiung Veteran General Hospital, Kaohsiung, Taiwan
| | - Jack P Wang
- Division of Gastroenterology, Department of Internal Medicine, Taipei City Hospital, Renai Branch, Taipei, Taiwan
| | - Hsiu-Po Wang
- Department of Internal Medicine, National Taiwan University Hospital, National Taiwan University, College of Medicine, Taipei, Taiwan.
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21
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Patel B, Klazynski B, Arora SP. High-Grade Large Cell Neuroendocrine Carcinoma: Case Report and Literature Review. J Gastrointest Cancer 2020; 51:341-343. [PMID: 31254245 DOI: 10.1007/s12029-019-00267-0] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/27/2022]
Affiliation(s)
- Bindiya Patel
- Mays Cancer Center, University of Texas Health San Antonio, 7979 Wurzbach Rd, MC8026, San Antonio, TX, 78229, USA
| | - Brian Klazynski
- Mays Cancer Center, University of Texas Health San Antonio, 7979 Wurzbach Rd, MC8026, San Antonio, TX, 78229, USA
| | - Sukeshi Patel Arora
- Mays Cancer Center, University of Texas Health San Antonio, 7979 Wurzbach Rd, MC8026, San Antonio, TX, 78229, USA.
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22
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Yuan G, Zhan C, Zhu D, Xie H, Wei T, Lu T, Yang Y, Zhu Y, Wang Q. Population-based analysis of esophageal large cell neuroendocrine carcinoma between 2004 and 2015. J Thorac Dis 2020; 11:5480-5488. [PMID: 32030267 DOI: 10.21037/jtd.2019.11.34] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/17/2022]
Abstract
Background Esophageal large cell neuroendocrine carcinoma (ELCNC) seems a rarely gastrointestinal malignancy. By far, its clinicopathological characteristics and prognosis have not been deeply studied. Methods The data of patients having ELCNC was extracted from the Surveillance, Epidemiology, and End Results (SEER) database, then assessed and compared with information from patients with esophageal small cell neuroendocrine carcinoma (ESCNC) or esophageal squamous cell carcinoma (ESCC). We used univariate and multivariate analyses to accurately detect independent prognostic factors. Results The data of 36 patients for ELCNC were obtained between 2004 and 2015. Compared with patients with ESCNC and ESCC, the mean survival time of ECLNC patients was worse than those with ESCC, while similar to ESCNC. Thus, ELCNC had significantly different clinicopathological characteristics compared to ESCNC and ESCC. Univariate and multivariate analyses revealed that age (P=0.001) and M stage (P=0.004) were independent prognostic factors. Conclusions ELCNC is a rare subtype of esophageal neuroendocrine carcinoma. The clinicopathological features differ from those of other esophageal carcinomas. Prognosis may be closely related to age and M stage.
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Affiliation(s)
- Guangda Yuan
- Department of Thoracic Surgery, The Affiliated Suzhou Hospital of Nanjing Medical University, Suzhou 215001, China
| | - Cheng Zhan
- Department of Thoracic Surgery, Zhongshan Hospital of Fudan University, Shanghai 200032, China
| | - Donglin Zhu
- Department of Thoracic Surgery, The Affiliated Suzhou Hospital of Nanjing Medical University, Suzhou 215001, China
| | - Hongya Xie
- Department of Thoracic Surgery, The Affiliated Suzhou Hospital of Nanjing Medical University, Suzhou 215001, China
| | - Tengteng Wei
- Department of Thoracic Surgery, The Affiliated Suzhou Hospital of Nanjing Medical University, Suzhou 215001, China
| | - Tao Lu
- Department of Thoracic Surgery, Zhongshan Hospital of Fudan University, Shanghai 200032, China
| | - Yong Yang
- Department of Thoracic Surgery, The Affiliated Suzhou Hospital of Nanjing Medical University, Suzhou 215001, China
| | - Yimeng Zhu
- Department of Thoracic Surgery, The Affiliated Suzhou Hospital of Nanjing Medical University, Suzhou 215001, China
| | - Qun Wang
- Department of Thoracic Surgery, Zhongshan Hospital of Fudan University, Shanghai 200032, China
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23
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Zhang G, Wu B, Wang X, Li J. A competing-risks nomogram and recursive partitioning analysis for cause-specific mortality in patients with esophageal neuroendocrine carcinoma. Dis Esophagus 2019; 32:5304731. [PMID: 30715226 DOI: 10.1093/dote/doy129] [Citation(s) in RCA: 8] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/16/2018] [Indexed: 12/11/2022]
Abstract
The objective of this study is to estimate the probability of cause-specific mortality using a competing-risks nomogram and recursive partitioning analysis in a large population-based cohort of patients with esophageal neuroendocrine carcinoma. The surveillance, epidemiology and end results database was used to identify 162 patients diagnosed with esophageal neuroendocrine carcinoma from 1998 to 2014. We estimated a cumulative incidence function for cause-specific mortality. A nomogram was constructed by using a proportional subdistribution hazard model, validated using bootstrap cross-validation, and evaluated with a decision curve analysis to assess its clinical utility. Finally, we performed risk stratification using a recursive partitioning analysis to divide patients with esophageal neuroendocrine carcinoma into clinically useful prognostic groups. Tumor location, distant metastasis, surgery, radiotherapy, and chemotherapy were significantly associated with cause-specific mortality. The calibration plots demonstrated good concordance between the predicted and actual outcomes. The discrimination performance of a Fine-Gray model was evaluated by using the c-index, which was 0.723 for cause-specific mortality. The decision curve analysis ranged from 0.268 to 0.968 for the threshold probability at which the risk model provided net clinical benefits relative to hypothetical all-screening and no-screening scenarios. The risk groups stratified by a recursive partitioning analysis allowed significant distinction between cumulative incidence curves. We determined the probability of cause-specific mortality in patients with esophageal neuroendocrine carcinoma and developed a nomogram and recursive partitioning analysis stratification system based on a competing-risks model. The nomogram and recursive partitioning analysis appear to be suitable for risk stratification of cause-specific mortality in patients with esophageal neuroendocrine carcinoma and will help clinicians to identify patients at increased risk of cause-specific mortality to guide treatment and surveillance decisions.
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Affiliation(s)
- G Zhang
- Department of Thoracic Surgery, First Affiliated Hospital of Zhengzhou University, Zhengzhou, Henan Province, China
| | - B Wu
- Department of Thoracic Surgery, First Affiliated Hospital of Zhengzhou University, Zhengzhou, Henan Province, China
| | - X Wang
- Department of Thoracic Surgery, First Affiliated Hospital of Zhengzhou University, Zhengzhou, Henan Province, China
| | - J Li
- Department of Thoracic Surgery, First Affiliated Hospital of Zhengzhou University, Zhengzhou, Henan Province, China
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24
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Ye L, Lu H, Wu L, Zhang L, Shi H, Wu HM, Tu P, Li DM, Wang FY. The clinicopathologic features and prognosis of esophageal neuroendocrine carcinomas: a single-center study of 53 resection cases. BMC Cancer 2019; 19:1234. [PMID: 31852479 PMCID: PMC6921601 DOI: 10.1186/s12885-019-6420-8] [Citation(s) in RCA: 12] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/08/2019] [Accepted: 12/01/2019] [Indexed: 01/08/2023] Open
Abstract
Background Esophageal neuroendocrine carcinomas (NECs) are exceedingly rare and poorly understood. The aims of the retrospective study were to delineate the clinicopathologic features and prognosis of patients with the disease. Methods We performed a retrospective study containing 53 patients of esophageal NECs in our center from 2002 through 2018. Patients were assigned to the pure esophageal NECs group and the esophageal NECs mixed with squamous carcinoma and/or esophageal adenocarcinoma (MiNECs) group. Demographic, clinical, pathologic and prognostic factors were recorded and analyzed. Results Of the 53 patients, elderly male patients were predominant. Dysphagia was the most common symptom (45/53, 84.9%). Most tumors were centered in the middle esophagus (36/53,67.9%).Ulcerated appearance was frequently seen in the pure NECs (56.8%), and the tumors in the MiNECs group mostly represented elevated types (57.9%). Synaptophysin (38/45, 84.4%), chromogranin A (21/38, 55.3%) and CD56(23/27, 85.2%) have been proven to be positive markers for NECs. Most patients (46/53, 86.8%) received surgery combined with chemotherapy. Though the pathologic stages were alike (P = 0.129), the median survival time was 3.53 years for the pure NECs group and 7 years for the MiNECs group. In multivariate analysis, pathologic stage (RR = 1.938, P = 0.045) and age (RR = 2.410, P = 0.028) were independent prognostic factors for patients with MiNECs. The prognosis of patients with pure NECs was independent from any factors. Conclusions Careful endoscopic examination could help distinguish pure NECs from MiNECs. NECs were aggressive, but a relative better prognosis for patients with MiNECs. Surgery should be performed if applicable, and chemotherapy might be helpful.
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Affiliation(s)
- Lei Ye
- Department of Gastroenterology and Hepatology, Jinling Hospital, Medical School of Nanjing University, 305 Zhongshan East Road, Nanjing, Jiangsu Province, 210002, China
| | - Heng Lu
- Department of Gastroenterology and Hepatology, Jinling Hospital, Medical School of Nanjing University, 305 Zhongshan East Road, Nanjing, Jiangsu Province, 210002, China
| | - Lin Wu
- Department of Gastroenterology and Hepatology, Jinling Hospital, Medical School of Nanjing University, 305 Zhongshan East Road, Nanjing, Jiangsu Province, 210002, China
| | - Lei Zhang
- Department of cardiothoracic surgery, Jinling Hospital, Medical School of Nanjing University, 305 Zhongshan East Road, Nanjing, Jiangsu Province, 210002, China
| | - Hui Shi
- Department of Gastroenterology and Hepatology, Jinling Hospital, Medical School of Nanjing University, 305 Zhongshan East Road, Nanjing, Jiangsu Province, 210002, China
| | - Hui Min Wu
- Department of Gastroenterology and Hepatology, Jinling Hospital, Medical School of Nanjing University, 305 Zhongshan East Road, Nanjing, Jiangsu Province, 210002, China
| | - Pin Tu
- Department of Pathology, Jinling Hospital, Medical School of Nanjing University, 305 Zhongshan East Road, Nanjing, Jiangsu Province, 210002, China
| | - De Min Li
- Department of cardiothoracic surgery, Jinling Hospital, Medical School of Nanjing University, 305 Zhongshan East Road, Nanjing, Jiangsu Province, 210002, China.
| | - Fang Yu Wang
- Department of Gastroenterology and Hepatology, Jinling Hospital, Medical School of Nanjing University, 305 Zhongshan East Road, Nanjing, Jiangsu Province, 210002, China.
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25
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Chen YT, Xie JY, Sun Q, Mo WJ. Novel drug candidates for treating esophageal carcinoma: A study on differentially expressed genes, using connectivity mapping and molecular docking. Int J Oncol 2018; 54:152-166. [PMID: 30387840 PMCID: PMC6254996 DOI: 10.3892/ijo.2018.4618] [Citation(s) in RCA: 10] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/08/2018] [Accepted: 10/23/2018] [Indexed: 12/14/2022] Open
Abstract
Patients with esophageal carcinoma (ESCA) have a poor prognosis and high mortality rate. Although standard therapies have had effect, there is an urgent requirement to develop novel options, as increasing drug tolerance has been identified in clinical practice. In the present study, differentially expressed genes (DEGs) of ESCA were identified in The Cancer Genome Atlas and Genotype-Tissue Expression databases. Functional and protein-protein interaction (PPI) analyses were performed. The Connectivity Map (CMAP) was selected to predict drugs for the treatment of ESCA, and their target genes were acquired from the Search Tool for Interactions of Chemicals (STITCH) by uploading the Simplified Molecular-Input Line-Entry System structure. Additionally, significant target genes and ESCA-associated hub genes were extracted using another PPI analysis, and the corresponding drugs were added to construct a network. Furthermore, the binding affinity between predicted drug candidates and ESCA-associated hub genes was calculated using molecular docking. Finally, 827 DEGs (|log2 fold-change|≥2; q-value <0.05), which are principally involved in protein digestion and absorption (P<0.005), the plasminogen-activating cascade (P<0.01), as well as the ‘biological regulation’ of the Biological Process, ‘membrane’ of the Cellular Component and ‘protein binding’ of the Molecular Function categories, were obtained. Additionally, 11 hub genes were obtained from the PPI network (all degrees ≥30). Furthermore, the 15 first screen drugs were extracted from CMAP (score <−0.85) and the 9 second screen drugs with 70 target genes were extracted from STITCH. Furthermore, another PPI analysis extracted 51 genes, and apigenin, baclofen, Prestwick-685, menadione, butyl hydroxybenzoate, gliclazide and valproate were selected as drug candidates for ESCA. Those molecular docking results with a docking score of >5.52 indicated the significance of apigenin, Prestwick-685 and menadione. The results of the present study may lead to novel drug candidates for ESCA, among which Prestwick-685 and menadione were identified to be significant new drug candidates.
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Affiliation(s)
- Yu-Ting Chen
- Department of Pathology, First Affiliated Hospital of Guangxi Medical University, Nanning, Guangxi 530021, P.R. China
| | - Jia-Yi Xie
- Department of Pathology, First Affiliated Hospital of Guangxi Medical University, Nanning, Guangxi 530021, P.R. China
| | - Qi Sun
- Department of Pathology, First Affiliated Hospital of Guangxi Medical University, Nanning, Guangxi 530021, P.R. China
| | - Wei-Jia Mo
- Department of Pathology, First Affiliated Hospital of Guangxi Medical University, Nanning, Guangxi 530021, P.R. China
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26
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He Y, Xu W, Xiao Y, Pan L, Chen G, Tang Y, Zhou J, Wu J, Zhu W, Zhang S, Cao J. Overexpression of Peroxiredoxin 6 (PRDX6) Promotes the Aggressive Phenotypes of Esophageal Squamous Cell Carcinoma. J Cancer 2018; 9:3939-3949. [PMID: 30410598 PMCID: PMC6218759 DOI: 10.7150/jca.26041] [Citation(s) in RCA: 18] [Impact Index Per Article: 2.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/13/2018] [Accepted: 09/04/2018] [Indexed: 12/11/2022] Open
Abstract
Esophageal squamous cell carcinoma (ESCC) is one of the most common malignancies. Peroxiredoxin 6 (PRDX6), a member of peroxidase superfamily, has a function of eliminating the reactive oxygen species (ROS), and participates in development of multiple diseases, including tumors. The purpose of this study was to investigate the expression of PRDX6 in normal and cancerous esophageal tissues and to characterize its role in ESCC progression. We found significantly higher expression of PRDX6 in ESCC tissues than in normal esophageal tissues or tumor-adjacent tissues and that the PRDX6 expression level was positively correlated with the proliferation-related markers. In ESCC cells, PRDX6 distribution was more pronounced in the nucleus region. PRDX6 overexpression by an adenovirus significantly promoted cell proliferation, migration and invasion in TE-1 and Eca-109 cells. Conversely, lentivirus-mediated knock-down of PRDX6 expression significantly reduced cell growth, colony formation and metastasis in ESCC cells. PRDX6 modulated the phosphorylation of Akt and Erk1/2, and the expression of MMP2. We also found that PRDX6 and Erk1/2 pathway were mutually regulated in ESCC cells. In addition, PRDX6 overexpression eliminated radiation-induced ROS and decreased consequent cell apoptosis, indicative of a role in radioresistance. Finally, the role of PRDX6 in promoting tumor growth was further confirmed in nude mice with ESCC xenografts. Taken together, we demonstrated that overexpression of PRDX6 promotes the progression of ESCC through Erk1/2, which provides a potential therapeutic target for human ESCC.
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Affiliation(s)
- Yan He
- School of Radiation Medicine and Protection, Medical College of Soochow University, Suzhou 215123, China.,State Key Laboratory of Radiation Medicine and Protection, Collaborative Innovation Center of Radiation Medicine of Jiangsu Higher Education Institutions, Soochow University, Suzhou 215123, China.,Suzhou Cancer Center Core Laboratory, Nanjing Medical University Affiliated Suzhou Hospital, Suzhou 215001, China
| | - Wanglei Xu
- School of Radiation Medicine and Protection, Medical College of Soochow University, Suzhou 215123, China.,State Key Laboratory of Radiation Medicine and Protection, Collaborative Innovation Center of Radiation Medicine of Jiangsu Higher Education Institutions, Soochow University, Suzhou 215123, China
| | - Yuji Xiao
- School of Radiation Medicine and Protection, Medical College of Soochow University, Suzhou 215123, China
| | - Lu Pan
- School of Radiation Medicine and Protection, Medical College of Soochow University, Suzhou 215123, China
| | - Guangxia Chen
- Department of Gastroenterology, First People's Hospital of Xuzhou, Xuzhou 221002, China
| | - Yiting Tang
- Department of Radiotherapy, Changzhou Tumor Hospital, Soochow University, Changzhou 213032, China
| | - Jundong Zhou
- Suzhou Cancer Center Core Laboratory, Nanjing Medical University Affiliated Suzhou Hospital, Suzhou 215001, China
| | - Jinchang Wu
- Suzhou Cancer Center Core Laboratory, Nanjing Medical University Affiliated Suzhou Hospital, Suzhou 215001, China
| | - Wei Zhu
- School of Radiation Medicine and Protection, Medical College of Soochow University, Suzhou 215123, China.,State Key Laboratory of Radiation Medicine and Protection, Collaborative Innovation Center of Radiation Medicine of Jiangsu Higher Education Institutions, Soochow University, Suzhou 215123, China
| | - Shuyu Zhang
- Department of Oncology, The Affiliated Changzhou No.2 People's Hospital of Nanjing Medical University, Changzhou 213100, China.,School of Radiation Medicine and Protection, Medical College of Soochow University, Suzhou 215123, China
| | - Jianping Cao
- School of Radiation Medicine and Protection, Medical College of Soochow University, Suzhou 215123, China.,State Key Laboratory of Radiation Medicine and Protection, Collaborative Innovation Center of Radiation Medicine of Jiangsu Higher Education Institutions, Soochow University, Suzhou 215123, China
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27
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Tomiyama T, Orino M, Nakamaru K, Tanaka T, Suzuki R, Okazaki T, Fukata N, Ando Y, Danbara N, Fukui T, Miyasaka C, Okazaki K. Esophageal Large-Cell Neuroendocrine Carcinoma with Inconsistent Response to Treatment in the Primary and Metastatic Lesions. Case Rep Gastroenterol 2018; 12:234-239. [PMID: 29928189 PMCID: PMC6006652 DOI: 10.1159/000489295] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/18/2018] [Accepted: 04/17/2018] [Indexed: 01/29/2023] Open
Abstract
Esophageal large-cell neuroendocrine carcinoma (NEC) is a rare malignant tumor that is characterized by high-grade malignancy and a poor prognosis. However, the rarity of esophageal NEC has prevented the development of an established treatment, and no reports have described a discrepancy in the effectiveness of cisplatin plus irinotecan between primary and metastatic lesions. A 43-year-old Japanese man was referred to our hospital with refractory epigastralgia. A previous gastrointestinal endoscopy had revealed a 50-mm type 2 tumor in the abdominal esophagus. The pathological findings indicated poorly differentiated squamous cell carcinoma. Contrast-enhanced computed tomography revealed a metastatic liver tumor. One cycle of fluorouracil and cisplatin was not effective, and endoscopy was repeatedly performed. The pathological findings indicated a large-cell malignant tumor with tumor cells that were positive for CD56, synaptophysin, and Ki-67 (> 80%). Based on a diagnosis of esophageal large-cell NEC with a metastatic liver tumor, the patient received cisplatin plus irinotecan biweekly. After 4 months, computed tomography revealed marked shrinkage of the metastatic tumor, but the patient complained of dysphagia. Endoscopy revealed enlargement of the primary tumor, which was then treated using radiotherapy plus fluorouracil and cisplatin. The primary tumor subsequently shrank, and the patient's symptoms were relieved, but the metastatic tumor grew. Thus, chemoradiotherapy could be an option for managing a primary esophageal large-cell NEC that does not respond to chemotherapy alone. However, the possibility of an inconsistent response to therapy in primary and metastatic lesions should be considered.
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Affiliation(s)
- Takashi Tomiyama
- Division of Gastroenterology and Hepatology, Third Department of Internal Medicine, Kansai Medical University, Osaka, Japan
| | - Masahiro Orino
- Division of Gastroenterology and Hepatology, Third Department of Internal Medicine, Kansai Medical University, Osaka, Japan
| | - Koh Nakamaru
- Division of Gastroenterology and Hepatology, Third Department of Internal Medicine, Kansai Medical University, Osaka, Japan
| | - Toshihiro Tanaka
- Division of Gastroenterology and Hepatology, Third Department of Internal Medicine, Kansai Medical University, Osaka, Japan
| | - Ryo Suzuki
- Division of Gastroenterology and Hepatology, Third Department of Internal Medicine, Kansai Medical University, Osaka, Japan
| | - Takashi Okazaki
- Division of Gastroenterology and Hepatology, Third Department of Internal Medicine, Kansai Medical University, Osaka, Japan
| | - Norimasa Fukata
- Division of Gastroenterology and Hepatology, Third Department of Internal Medicine, Kansai Medical University, Osaka, Japan
| | - Yugo Ando
- Division of Gastroenterology and Hepatology, Third Department of Internal Medicine, Kansai Medical University, Osaka, Japan
| | - Naoyuki Danbara
- Division of Gastroenterology and Hepatology, Third Department of Internal Medicine, Kansai Medical University, Osaka, Japan
| | - Toshiro Fukui
- Division of Gastroenterology and Hepatology, Third Department of Internal Medicine, Kansai Medical University, Osaka, Japan
| | - Chika Miyasaka
- Department of Pathology and Laboratory Medicine, Kansai Medical University, Osaka, Japan
| | - Kazuichi Okazaki
- Division of Gastroenterology and Hepatology, Third Department of Internal Medicine, Kansai Medical University, Osaka, Japan
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