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Zhang Y, Yang Y, Gao C, Zhao H, Zhou H. Lateral pelvic lymph nodes dissection of rectal neuroendocrine neoplasms: A prospective case-series and literature review. Surgery 2024; 176:1360-1365. [PMID: 39147667 DOI: 10.1016/j.surg.2024.07.004] [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: 05/05/2024] [Revised: 07/01/2024] [Accepted: 07/03/2024] [Indexed: 08/17/2024]
Abstract
BACKGROUND Rectal neuroendocrine neoplasms are relatively rare. Patients with rectal neuroendocrine neoplasms undergoing radical surgery have a higher rate of lymph node metastases. Robust evidence on the status of lateral pelvic lymph node metastases and the role of lateral pelvic lymph node dissection in those patients is lacking. This case-series study aimed to explore and address these issues. METHODS This single-center, prospective case series consecutively enrolled patients with biopsy-proven rectal neuroendocrine neoplasms in a tertiary referral hospital between June 2022 and January 2024. All eligible patients underwent laparoscopic total mesorectal excision surgery and bilateral lateral pelvic lymph node dissection under general anesthesia. The clinicopathologic features, surgical outcomes, and postoperative complications were presented. The last follow-up was conducted in March 2024. RESULTS A total of 11 patients with rectal neuroendocrine neoplasms-3 female and 8 male-were enrolled. The average age was 60.0 years (range, 53.5-65.5 years), and the median tumor size was 2.0 cm (range, 1.6-2.5 cm). Tumors invaded the muscularis propria in 7 patients. There were 3 cases of neuroendocrine tumor G1, 6 cases of neuroendocrine tumor G2, and 2 cases of neuroendocrine carcinoma. Among these patients, 11 (100.0%) had lymph node metastases, and 6 (54.5%) had lateral pelvic lymph node metastases. In addition, in 2 patients, only lateral pelvic lymph node metastases were observed, without involvement of the mesenteric lymph nodes. Five patients had tumors located on the left wall of the rectum, and only left-sided lateral pelvic lymph node metastases were observed. The other patient had both sides of lateral pelvic lymph node metastases due to circumferential growth of the tumor around the rectum. Anal preservation was achieved in all patients. The median operating time was 235.0 minutes (range, 210.5-335.5 minutes), and the median estimated blood loss was 50.0 mL (range, 45.0-75.0 mL). Two patients experienced postoperative dysuria and recovered spontaneously within 2-4 months after surgery. CONCLUSION On the basis of a prospective case series, we demonstrate, for the first time, the lateral pelvic lymph node metastasis status in patients with rectal neuroendocrine neoplasms requiring radical total mesorectal excision surgery. Simultaneous bilateral lateral pelvic lymph node dissection may be a feasible and beneficial procedure for preventing local recurrence in these patients due to the lack of definitive neoadjuvant or adjuvant therapy options.
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Affiliation(s)
- Yueyang Zhang
- Department of Colorectal Surgery, National Cancer Center/National Clinical Research Center for Cancer/Cancer Hospital, Chinese Academy of Medical Sciences (CAMS) and Peking Union Medical College (PUMC), Beijing, China
| | - Yi Yang
- Department of Hepatobiliary Surgery, National Cancer Center/National Clinical Research Center for Cancer/Cancer Hospital, Chinese Academy of Medical Sciences (CAMS) and Peking Union Medical College (PUMC), Beijing, China
| | - Changyuan Gao
- Department of Colorectal Surgery, National Cancer Center/National Clinical Research Center for Cancer/Cancer Hospital, Chinese Academy of Medical Sciences (CAMS) and Peking Union Medical College (PUMC), Beijing, China
| | - Hong Zhao
- Department of Hepatobiliary Surgery, National Cancer Center/National Clinical Research Center for Cancer/Cancer Hospital, Chinese Academy of Medical Sciences (CAMS) and Peking Union Medical College (PUMC), Beijing, China
| | - Haitao Zhou
- Department of Colorectal Surgery, National Cancer Center/National Clinical Research Center for Cancer/Cancer Hospital, Chinese Academy of Medical Sciences (CAMS) and Peking Union Medical College (PUMC), Beijing, China.
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2
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Li C, Yu M, Liu W, Zhang W, Jiang W, Zhang P, Zeng X, Di M, Liao X, Zheng Y, Xiong Z, Xia L, Sun Y, Zhang R, Zhong M, Lin G, Lin R, Tao K. Long-term outcomes of 1-2 cm rectal neuroendocrine tumors after local excision or radical resection: A population-based multicenter study. Heliyon 2024; 10:e28335. [PMID: 38571595 PMCID: PMC10988025 DOI: 10.1016/j.heliyon.2024.e28335] [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: 10/01/2023] [Revised: 03/14/2024] [Accepted: 03/15/2024] [Indexed: 04/05/2024] Open
Abstract
Objectives Studies on rectal neuroendocrine tumors (R-NETs) that are 1-2 cm in size are limited, and the optimal treatment for these tumors is not well established. Methods Data from patients with primary localized R-NETs 1-2 cm in size were retrospectively collected from 17 large-scale referral medical centers in China. Long-term prognosis, quality of life (QOL), and fecal incontinence were evaluated, and the effects of local excision (LE) or radical resection (RR) were elucidated using propensity score matching (PSM). Results A total of 272 patients were included in this study; 233 underwent LE, and the remaining 39 underwent RR. Patients in the LE group showed lower tumor location, fewer postoperative Clavien-Dindo III-V complications, more G1 tumors, and lower tumor stage. There were no significant differences in the relapse-free survival or overall survival (OS) between the LE and RR groups after PSM. Patients in the LE group reported superior physical, role, emotional, social, and cognitive functions, global QOL, and Wexner fecal incontinence scores compared with those in the RR group (all P < 0.050). Eighteen (6.6%) patients had lymph node metastases. Multivariable analysis revealed that tumor location (odds ratio [OR] = 3.19, 95% confidence interval [CI] 1.04-10.07, P = 0.010), neutrophil-to-lymphocyte ratio (NLR) > 1.80 (OR = 4.50, 1.46-15.89, P = 0.012), and T3-T4 (OR = 36.31, 95% CI 7.85-208.62, P < 0.001) were independent risk factor for lymph node metastasis. Conclusions R-NETs measuring 1-2 cm generally have a favorable prognosis, and there is no difference in postoperative survival between LE and RR. For patients without lymph node metastasis, LE should be the preferred choice; however, for patients with a higher tumor location, preoperative NLR >1.8 or T3/T4 tumors, RR should be considered.
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Affiliation(s)
- Chengguo Li
- Department of Gastrointestinal Surgery, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, 430022, China
| | - Minhao Yu
- Department of Gastrointestinal Surgery, Renji Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai, 200127, China
| | - Weizhen Liu
- Department of Gastrointestinal Surgery, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, 430022, China
| | - Wei Zhang
- Department of Colorectal Surgery, Shanghai Changhai Hospital, Naval Medical University, Shanghai, 200082, China
| | - Weizhong Jiang
- Department of Colorectal Surgery, Fujian Medical University Union Hospital, Fuzhou, 350001, China
| | - Peng Zhang
- Department of Gastrointestinal Surgery, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, 430022, China
| | - Xinyu Zeng
- Department of Gastrointestinal Surgery, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, 430022, China
| | - Maojun Di
- Department of Gastrointestinal Surgery, Taihe Hospital, Hubei University of Medicine, Shiyan, 442000, China
| | - Xiaofeng Liao
- Department of General Surgery, Xiangyang Central Hospital, Affiliated Hospital of Hubei University of Arts and Science. Institute of Oncology, Xiangyang Central Hospital, Affiliated Hospital of Hubei University of Arts and Science, Xiangyang, 441021, China
| | - Yongbin Zheng
- Department of Gastrointestinal Surgery, Renmin Hospital of Wuhan University, Wuhan, 430060, China
| | - Zhiguo Xiong
- Department of Gastrointestinal Surgery, Hubei Cancer Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, 430079, China
| | - Lijian Xia
- Department of Colorectal and Anal Surgery, The First Affiliated Hospital of Shandong First Medical University, Jinan, 250014, China
| | - Yueming Sun
- Department of Colorectal Surgery, Jiangsu Province Hospital, Nanjing Medical University, Nanjing, 210029, China
| | - Rui Zhang
- Department of Colorectal Cancer, Liaoning Cancer Hospital & Institute, Shenyang, 110042, China
| | - Ming Zhong
- Department of Gastrointestinal Surgery, Renji Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai, 200127, China
| | - Guole Lin
- Department of General Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, 100730, China
| | - Rong Lin
- Department of Gastroenterology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, 430022, China
| | - Kaixiong Tao
- Department of Gastrointestinal Surgery, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, 430022, China
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3
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Zheng X, Wu M, Li S, Er L, Deng H, Guo S, Liu Z. Clinicopathological characteristics of rectal multiple neuroendocrine neoplasms and literature review. BMC Surg 2023; 23:147. [PMID: 37264328 DOI: 10.1186/s12893-023-02050-2] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/08/2023] [Accepted: 05/21/2023] [Indexed: 06/03/2023] Open
Abstract
BACKGROUND There are only a few epidemiological reports available for reference. The clinicopathological features are not clear, so there is no consensus on treating rectal multiple neuroendocrine neoplasms. This study aims to summarize the clinicopathological characteristics and preliminarily discuss the clinical diagnosis and treatment of rectal multiple neuroendocrine neoplasms. METHODS This study retrospectively analyzed rectal neuroendocrine neoplasm patients diagnosed and treated at the Fourth Hospital of Hebei Medical University from February 2007 to May 2021. The clinicopathological characteristics of rectal multiple neuroendocrine neoplasms were summarized and analyzed in combination with 14 studies on rectal multiple neuroendocrine neoplasms. RESULTS The incidence of RM-NENs accounted for 3.8% of all R-NENs in this study. The number of tumors varied to some extent, the size of tumors was basically no more than 10 mm, and there were more G1 grade tumors. In the analysis of 46 cases with known lymph node metastasis, the difference in lymph node metastasis rate between the number of tumors < 8 and ≥ 8 was statistically significant (p = 0.002). CONCLUSIONS The incidence of rectal multiple neuroendocrine neoplasms accounted for 3.8% of all rectal neuroendocrine neoplasms. For rectal multiple neuroendocrine neoplasms, the lymph node metastasis rate was higher when the number of tumors was ≥ 8. The influence of the number of tumors on lymph node metastasis should be considered in the selection of treatment.
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Affiliation(s)
- Xiuli Zheng
- Department of Endoscopy, Fourth Hospital of Hebei Medical University, No. 12 Jiankang Road, Chang'an District, Shijiazhuang, 050000, Hebei, China
| | - Mingli Wu
- Department of Endoscopy, Fourth Hospital of Hebei Medical University, No. 12 Jiankang Road, Chang'an District, Shijiazhuang, 050000, Hebei, China.
| | - Shengmian Li
- Department of Gastroenterology, Fourth Hospital of Hebei Medical University, No.12 Jiankang Road, Chang'an District, Shijiazhuang, 050000, Hebei, China
| | - Limian Er
- Department of Endoscopy, Fourth Hospital of Hebei Medical University, No. 12 Jiankang Road, Chang'an District, Shijiazhuang, 050000, Hebei, China
| | - Huiyan Deng
- Department of Pathology, Fourth Hospital of Hebei Medical University, No. 12 Jiankang Road, Chang'an District, Shijiazhuang, 050000, Hebei, China
| | - Shuo Guo
- Department of Endoscopy, Fourth Hospital of Hebei Medical University, No. 12 Jiankang Road, Chang'an District, Shijiazhuang, 050000, Hebei, China
| | - Zhihuan Liu
- Department of Endoscopy, Fourth Hospital of Hebei Medical University, No. 12 Jiankang Road, Chang'an District, Shijiazhuang, 050000, Hebei, China
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Isolated diffuse intestinal ganglioneuromatosis presented as a redundant sigmoid colon: a case report. Ann Med Surg (Lond) 2023; 85:219-224. [PMID: 36845810 PMCID: PMC9949766 DOI: 10.1097/ms9.0000000000000195] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/18/2022] [Accepted: 12/25/2022] [Indexed: 02/28/2023] Open
Abstract
Diffuse intestinal ganglioneuromtosis is a benign tumor of the enteric nervous system, that almost always occurs in children with systemic syndromes. Whereas isolated cases in adults are exceedingly rare. Case Presentation A 38-year-old man presented with refractory chronic constipation. An abdominal computed tomography scan revealed a redundant sigmoid colon, then he underwent a sigmoid colectomy. Histopathologic examination showed diffuse ganglioneuromatosis. However, the patient was in good health 18 months after surgery. Clinical Discussion Intestinal ganglioneuromas commonly occur in children with systemic syndromes such as multiple endocrine neoplasia type 2B and neurofibromatosis type 1. The most frequent symptoms are abdominal discomfort, constipation, ileus, weight loss, appendicitis, and obstruction in more severe cases. surgical resection is the standard management in diffuse ganglioneuromatosis. Conclusion Although diffuse ganglioneuromatosis is uncommon, it should be considered in patients with refractory constipation.
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Pang S, Zong Y, Zhang K, Zhao H, Wang Y, Wang J, Liu C, Wu Y, Li P. Multiple rectal neuroendocrine tumors: An analysis of 15 cases and literature review. Front Oncol 2022; 12:996306. [PMID: 36185313 PMCID: PMC9515498 DOI: 10.3389/fonc.2022.996306] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/17/2022] [Accepted: 08/24/2022] [Indexed: 12/07/2022] Open
Abstract
Multiple neuroendocrine tumors (M-NETs) are rare in the rectum and there is no consensus on their characteristics and treatments. Here, we report 15 cases of rectal M-NETs and review the previous literature. We discuss the clinical characteristics, endoscopic features and pathological features of rectal M-NETs, aiming to analyze the treatments and follow-up strategies in combination with these characteristics. We retrospectively reviewed and analyzed the data of 15 patients with rectal M-NETs who were diagnosed and treated at Beijing Friendship Hospital, Capital Medical University. Their clinical data, endoscopic findings, pathological features and treatments were analyzed. Follow-up evaluations and literature review were performed. In all, 14 male (93.3%) and 1 female (6.7%) were recruited. The average age at diagnosis was 55.7 years. The clinical manifestations include asymptomatic in 9 patients (60.0%), defecation habits changes in 2 patients (13.3%), anal distension in 2 patients (13.3%), and abdominal distension in 2 patient (13.3%). The largest tumor diameter ≤10mm was found in 13 patients (86.7%) and >10mm in 2 patients (13.3%). All of the lesions originated from the mucous or submucosa layer. WHO grades were all NET G1. The number of tumors diagnosed by pathology in 13 patients was consistent with that observed by endoscopy, while more lesions were observed by pathology than endoscopy in two patients. Lymph node metastasis occurred in 1 patient (6.7%), and vascular or lymphatic invasion occurred in 9 patients (60.0%). Among the 13 patients with the largest tumor diameter being ≤10mm, lymphovascular invasion occurred in 8 patients (61.5%). And among the 2 patients with the largest tumor diameter of >10mm, lymphovascular invasion occurred in 1 patient (50.0%). 14 patients underwent endoscopic resection and 1 underwent surgical excision. Postoperative follow-up was achieved in 13 patients and no recurrence or metastasis was found. The true number of rectal M-NETs may be more than seen under endoscopy. Rectal M-NETs is associated with a high risk of metastasis; therefore, treatment and surveillance strategies should be more radical than single lesion.
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Affiliation(s)
- Shu Pang
- Department of General Practice, Beijing Friendship Hospital, Capital Medical University, Beijing, China
| | - Ye Zong
- Department of Gastroenterology, Beijing Friendship Hospital, Capital Medical University, Beijing, China
| | - Kun Zhang
- Department of Pathology, Beijing Friendship Hospital, Capital Medical University, Beijing, China
| | - Haiying Zhao
- Department of Gastroenterology, Beijing Friendship Hospital, Capital Medical University, Beijing, China
| | - Yongjun Wang
- Department of Gastroenterology, Beijing Friendship Hospital, Capital Medical University, Beijing, China
| | - Junxiong Wang
- Department of Gastroenterology, Beijing Friendship Hospital, Capital Medical University, Beijing, China
| | - Chuntao Liu
- Department of Gastroenterology, Beijing Friendship Hospital, Capital Medical University, Beijing, China
| | - Yongdong Wu
- Department of Gastroenterology, Beijing Friendship Hospital, Capital Medical University, Beijing, China
- *Correspondence: Yongdong Wu, ; Peng Li,
| | - Peng Li
- Department of Gastroenterology, Beijing Friendship Hospital, Capital Medical University, Beijing, China
- *Correspondence: Yongdong Wu, ; Peng Li,
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6
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Well-differentiated neuroendocrine tumour arising from diffuse ganglioneuromatosis: first reported case. Pathology 2021; 54:488-490. [PMID: 34711414 DOI: 10.1016/j.pathol.2021.08.005] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/22/2021] [Accepted: 08/02/2021] [Indexed: 11/20/2022]
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7
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Mohammed Kheir H, Dayoub M, Haidar N, Mansour H, Omran A, Ibrahim A, Alshehabi Z. Incidental Delayed Diagnosis of Isolated Diffuse Ganglioneuromatosis Caused the Death of a 6-Year-Old Boy: Case Report. CLINICAL MEDICINE INSIGHTS-CASE REPORTS 2021; 14:11795476211049864. [PMID: 34629921 PMCID: PMC8493302 DOI: 10.1177/11795476211049864] [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: 07/30/2021] [Accepted: 09/01/2021] [Indexed: 01/17/2023]
Abstract
Intestinal diffuse ganglioneuromatosis is a rare, benign, neoplastic condition characterized by disseminated proliferation of neural elements. It has an established association with several systemic disorders including: multiple endocrine neoplasia IIB (MEN IIB) and von Recklinghausen's disease. However, isolated GNs are very rare and sporadic. We report a case of a 6-year-old patient was referred to the emergency department due to a colon perforation during endoscopy. He then underwent sigmoidectomy and the pathologic studies revealed prominent signs of diffuse ganglioneuromatosis. Unfortunately, the patient died the day after because of the late diagnosis and treatment. Such presentation emphasizes the importance of considering diffuse ganglioneuromatosis, among other causes, in patients with intractable GI symptoms despite medical treatment.
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Affiliation(s)
- Haya Mohammed Kheir
- Department of Pathology, Tishreen University Hospital, Latakia, Syria.,Cancer Research Center, Tishreen University Hospital, Latakia, Syria
| | - Mouhannad Dayoub
- Cancer Research Center, Tishreen University Hospital, Latakia, Syria
| | - Nour Haidar
- Faculty of Medicine, Tishreen University, Latakia, Syria
| | - Hanin Mansour
- Department of Pediatrics, Tishreen University Hospital, Latakia, Syria
| | - Ammar Omran
- Department of Pediatric Surgery, Tishreen University Hospital, Latakia Syria
| | - Ali Ibrahim
- Department of Pediatrics, Tishreen University Hospital, Latakia, Syria
| | - Zuheir Alshehabi
- Department of Pathology, Tishreen University Hospital, Latakia, Syria.,Cancer Research Center, Tishreen University Hospital, Latakia, Syria
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8
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Merola G, Bracale U, Sodo M, Andreuccetti J, Pignata G. Laparoscopic treatment of mixed adenoneuroendocrine cancer of right colon: case report and review of literature. Chirurgia (Bucur) 2020. [DOI: 10.23736/s0394-9508.19.05003-4] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/08/2022]
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9
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Suzuki S, Kawakami H, Miike T, Yamamoto S, Abe H, Shimoda K, Ashizuka S, Inatsu H, Kubota Y, Ban T, Yorita K, Kataoka H. Single Rectal Neuroendocrine Tumor Associated with Multiple Endocrine Cell Micronests. Intern Med 2020; 59:619-623. [PMID: 31666465 PMCID: PMC7086335 DOI: 10.2169/internalmedicine.3582-19] [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] [Indexed: 11/06/2022] Open
Abstract
Although a few reports of neuroendocrine tumor (NET) in the stomach or appendix with surrounding micronests have been published, cases of rectal NET are rare. We herein report a unique case of a patient with single rectal NET treated endoscopically. A pathological examination revealed multiple endocrine cell micronests (ECMs) in the submucosal layer around the main NET lesion. Neither lymph node metastasis nor distant metastasis in computed tomography was observed six years after the treatment. Because case reports of multiple ECM are very rare, the significance of malignancy is unclear. It therefore appears to be necessary to accumulate similar cases.
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Affiliation(s)
- Sho Suzuki
- Department of Gastroenterology and Hematology, Center for Digestive Disease and Division of Endoscopy, University of Miyazaki Hospital, Japan
- Department of Gastroenterology and Hepatology, Department of Internal Medicine, Faculty of Medicine, University of Miyazaki, Japan
| | - Hiroshi Kawakami
- Department of Gastroenterology and Hematology, Center for Digestive Disease and Division of Endoscopy, University of Miyazaki Hospital, Japan
- Division of Gastroenterology and Hepatology, Department of Internal Medicine, Faculty of Medicine, University of Miyazaki, Japan
| | - Tadashi Miike
- Department of Gastroenterology and Hematology, Center for Digestive Disease and Division of Endoscopy, University of Miyazaki Hospital, Japan
- Department of Gastroenterology and Hepatology, Department of Internal Medicine, Faculty of Medicine, University of Miyazaki, Japan
| | - Shojiro Yamamoto
- Department of Gastroenterology and Hematology, Center for Digestive Disease and Division of Endoscopy, University of Miyazaki Hospital, Japan
- Department of Gastroenterology and Hepatology, Department of Internal Medicine, Faculty of Medicine, University of Miyazaki, Japan
| | - Hiroo Abe
- Department of Gastroenterology and Hematology, Center for Digestive Disease and Division of Endoscopy, University of Miyazaki Hospital, Japan
- Department of Gastroenterology and Hepatology, Department of Internal Medicine, Faculty of Medicine, University of Miyazaki, Japan
| | - Kazuya Shimoda
- Department of Gastroenterology and Hepatology, Department of Internal Medicine, Faculty of Medicine, University of Miyazaki, Japan
| | - Shinya Ashizuka
- Department of Gastroenterology and Hematology, Center for Digestive Disease and Division of Endoscopy, University of Miyazaki Hospital, Japan
| | - Haruhiko Inatsu
- Department of Gastroenterology and Hematology, Center for Digestive Disease and Division of Endoscopy, University of Miyazaki Hospital, Japan
| | - Yoshimasa Kubota
- Department of Gastroenterology and Hematology, Center for Digestive Disease and Division of Endoscopy, University of Miyazaki Hospital, Japan
- Division of Gastroenterology and Hepatology, Department of Internal Medicine, Faculty of Medicine, University of Miyazaki, Japan
| | - Tesshin Ban
- Department of Gastroenterology and Hematology, Center for Digestive Disease and Division of Endoscopy, University of Miyazaki Hospital, Japan
- Division of Gastroenterology and Hepatology, Department of Internal Medicine, Faculty of Medicine, University of Miyazaki, Japan
| | - Kenji Yorita
- Department of Diagnostic Pathology, Japanese Red Cross Kochi Hospital, Japan
- Section of Oncopathology and Regenerative Biology, Department of Pathology, Faculty of Medicine, University of Miyazaki, Japan
| | - Hiroaki Kataoka
- Section of Oncopathology and Regenerative Biology, Department of Pathology, Faculty of Medicine, University of Miyazaki, Japan
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10
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Xie R, Fu KI, Chen SM, Tuo BG, Wu HC. Neurofibromatosis type 1-associated multiple rectal neuroendocrine tumors: A case report and review of the literature. World J Gastroenterol 2018; 24:3806-3812. [PMID: 30197486 PMCID: PMC6127664 DOI: 10.3748/wjg.v24.i33.3806] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/17/2018] [Revised: 07/12/2018] [Accepted: 07/21/2018] [Indexed: 02/06/2023] Open
Abstract
Neurofibromatosis type 1 (NF-1) is commonly associated with benign or malignant tumors in both the central and peripheral nervous systems. However, rare cases of NF-1-associated multiple rectal neuroendocrine tumors have been reported. This report describes a case of a 39 year old female with NF-1 and intermittent hematochezia as a primary symptom. Physical examination showed multiple subcutaneous nodules and café au lait spots with obvious scoliosis of the back. Imaging examinations and colonoscopy found malformation of the left external iliac vein and multiple gray-yellow nodules with varying sizes and shapes in the rectal submucosal layer. Histological and immunohistochemical results suggested multiple rectal neuroendocrine tumors, a rare disease with few appreciable symptoms and a particularly poor prognosis. The patient with NF-1 presented here had not only multiple rectal neuroendocrine neoplasms but also vascular malformations, scoliosis and other multiple system lesions. This case therefore contributes to improving clinical understanding, diagnosis and treatment of related complications for patients with NF-1 who present with associated medical conditions.
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Affiliation(s)
- Rui Xie
- Department of Gastroenterology, Affiliated Hospital to Zunyi Medical College, Zunyi 563003, Guizhou Province, China
| | - Kuang-I Fu
- Department of Gastroenterology, Affiliated Hospital to Zunyi Medical College, Zunyi 563003, Guizhou Province, China
- Department of Endoscopy, Kanma Memorial Hospital, Tokyo 3250046, Japan
| | - Shao-Min Chen
- Department of Gastroenterology, Affiliated Hospital to Zunyi Medical College, Zunyi 563003, Guizhou Province, China
| | - Bi-Guang Tuo
- Department of Gastroenterology, Affiliated Hospital to Zunyi Medical College, Zunyi 563003, Guizhou Province, China
| | - Hui-Chao Wu
- Department of Gastroenterology, Affiliated Hospital to Zunyi Medical College, Zunyi 563003, Guizhou Province, China
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11
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Park SS, Han N, Lee J, Chang HJ, Oh JH, Sohn DK. Multiple small, rectal neuroendocrine tumors with numerous micronests. J Dig Dis 2018; 19:572-575. [PMID: 29989305 DOI: 10.1111/1751-2980.12645] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/10/2018] [Revised: 04/23/2018] [Accepted: 07/05/2018] [Indexed: 12/11/2022]
Affiliation(s)
- Sung Sil Park
- Center for Colorectal Cancer, Research Institute and Hospital, National Cancer Center, Goyang, Korea
| | - Nayoung Han
- Department of Pathology, Research Institute and Hospital, National Cancer Center, Goyang, Korea
| | - Jihyeon Lee
- Department of Pathology, Research Institute and Hospital, National Cancer Center, Goyang, Korea
| | - Hee Jin Chang
- Center for Colorectal Cancer, Research Institute and Hospital, National Cancer Center, Goyang, Korea.,Department of Pathology, Research Institute and Hospital, National Cancer Center, Goyang, Korea
| | - Jae Hwan Oh
- Center for Colorectal Cancer, Research Institute and Hospital, National Cancer Center, Goyang, Korea
| | - Dae Kyung Sohn
- Center for Colorectal Cancer, Research Institute and Hospital, National Cancer Center, Goyang, Korea
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12
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Diffuse Gastric Ganglioneuromatosis: Novel Presentation of PTEN Hamartoma Syndrome-Case Report and Review of Gastric Ganglioneuromatous Proliferations and a Novel PTEN Gene Mutation. Case Rep Med 2018; 2018:4319818. [PMID: 29770149 PMCID: PMC5889855 DOI: 10.1155/2018/4319818] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/22/2017] [Accepted: 02/26/2018] [Indexed: 02/01/2023] Open
Abstract
Gastrointestinal ganglioneuromatous proliferations are rare, most often found in the colon, and are three types: polypoid ganglioneuromas, ganglioneuromatous polyposis, and diffuse ganglioneuromatosis. We present a case of diffuse ganglioneuromatosis in the posterior gastric wall in a nine-year-old female. To our knowledge, this is the first reported case of diffuse ganglioneuromatosis located in the stomach. Only six cases of gastric ganglioneuromatous proliferations have previously been reported, two in English and none were diffuse ganglioneuromatosis. A diagnosis of diffuse ganglioneuromatosis is relevant for patient care because, unlike sporadic polypoid ganglioneuromas or ganglioneuromatous polyposis, most are syndromic. Diffuse ganglioneuromatosis is commonly associated with neurofibromatosis type 1, multiple endocrine neoplasia type 2b, and Cowden Syndrome, one of the phenotypes of PTEN hamartoma tumor syndrome. The patient had the noted gastric diffuse ganglioneuromatosis, as well as other major and minor criteria for Cowden syndrome. Genetic testing revealed a novel frameshift mutation in the PTEN gene in the patient, her father, paternal aunt, and the aunt's son who is a paternal first cousin of the patient.
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Kimura K, Yamano T, Matsuda I, Babaya A, Hamanaka M, Kobayashi M, Tsukamoto K, Noda M, Hirota S, Tomita N. Multiple Rectal Neuroendocrine Tumors with Recurrence of Inguinal Lymph Nodes. ACTA ACUST UNITED AC 2018. [DOI: 10.5833/jjgs.2017.0031] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/11/2022]
Affiliation(s)
- Kei Kimura
- Division of Lower GI Surgery, Department of Surgery, Hyogo College of Medicine
| | - Tomoki Yamano
- Division of Lower GI Surgery, Department of Surgery, Hyogo College of Medicine
| | - Ikuo Matsuda
- Department of Surgical Pathology, Hyogo College of Medicine for Preparation of Pathological Specimen
| | - Akihito Babaya
- Division of Lower GI Surgery, Department of Surgery, Hyogo College of Medicine
| | - Michiko Hamanaka
- Division of Lower GI Surgery, Department of Surgery, Hyogo College of Medicine
| | - Masayoshi Kobayashi
- Division of Lower GI Surgery, Department of Surgery, Hyogo College of Medicine
| | - Kiyoshi Tsukamoto
- Division of Lower GI Surgery, Department of Surgery, Hyogo College of Medicine
| | - Masafumi Noda
- Division of Lower GI Surgery, Department of Surgery, Hyogo College of Medicine
| | - Seiichi Hirota
- Department of Surgical Pathology, Hyogo College of Medicine for Preparation of Pathological Specimen
| | - Naohiro Tomita
- Division of Lower GI Surgery, Department of Surgery, Hyogo College of Medicine
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Short- and Long-term Outcomes of Laparoscopic Total Mesenteric Excision for Neuroendocrine Tumors of the Rectum. Dis Colon Rectum 2017; 60:284-289. [PMID: 28177990 DOI: 10.1097/dcr.0000000000000745] [Citation(s) in RCA: 24] [Impact Index Per Article: 3.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/08/2023]
Abstract
BACKGROUND To our knowledge, no studies to date have assessed the short- and long-term outcomes of laparoscopic total mesenteric excision in patients with neuroendocrine tumors of the rectum. OBJECTIVE The purpose of this study was to investigate the short- and long-term outcomes of patients who underwent laparoscopic rectal resection plus total mesenteric excision for rectal neuroendocrine tumors at our institution. DESIGN This was a single center, retrospective study. SETTINGS The study was conducted at a tertiary care facility. PATIENTS Eight-two patients with neuroendocrine tumors who underwent rectal resection with total mesenteric excision, 77 laparoscopically, between June 2005 and August 2015 were included. INTERVENTIONS Laparoscopic rectal resection and total mesenteric excision were the study interventions. MAIN OUTCOME MEASURES Demographic characteristics and surgical and postoperative outcomes were measured. RESULTS Median tumor size was 8.8 mm (range, 3.0-35.0 mm); 63.6% of tumors were located in the lower rectum, with the median distance from the tumor to the anal verge being 50.0 mm (range, 20.0-130.0 mm). Anal preservation was achieved in all of the patients. Anastomotic leakage occurred in 5 patients (6.5%), but there were no deaths. Seventy-one patients (92.2%) had tumor invasion confined to the submucosa. Lymph node metastasis was present in 29 patients (37.7%), including 26 (33.8%) with perirectal and 5 (6.5%) with lateral lymph node metastasis. The median follow-up period in 59 patients was 42 months (range, 11-113 months), and the 3-year overall survival rate was 97.8%. LIMITATIONS The study was limited by its single-center, retrospective analysis. CONCLUSIONS Laparoscopic rectal resection with total mesenteric excision is safe in patients with rectal neuroendocrine tumors, with good short- and long-term outcomes. Because rectal neuroendocrine tumors are smaller and show superficial invasion, the rate of anal preservation may be high.
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Park CS, Lee SH, Kim SB, Kim KO, Jang BI. Multiple rectal neuroendocrine tumors: report of five cases. THE KOREAN JOURNAL OF GASTROENTEROLOGY 2016; 64:103-9. [PMID: 25168053 DOI: 10.4166/kjg.2014.64.2.103] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 12/20/2022]
Abstract
Carcinoids are slow growing neuroendocrine tumors (NET) originating in the enterochromaffin cells of the gastrointestinal tract. In previous studies, rectal NET comprised only about 1% of all anorectal neoplasms; however, the incidence of rectal NET has shown a recent increase. Typically, rectal NET presents as a single subepithelial nodule, and multicentricity of rectal NETs is rare, with reported incidence of 2-4.5%. Due to the rarity of multiple rectal NETs, there is no consensus or guidelines for treatment of multiple rectal NETs. However, NETs of the rectum that are less than 10 mm in diameter and do not infiltrate the muscularis propria, without distant metastasis, can be removed by endoscopy, as with solitary rectal NET. We encountered five cases of multiple rectal NETs which were treated successfully by endoscopy.
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Affiliation(s)
- Chan Seo Park
- Department of Internal Medicine, Yeungnam University College of Medicine, 170 Hyeonchung-ro, Nam-gu, Daegu 705-717, Korea
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Zhou JL, Lin GL, Zhao DC, Zhong GX, Qiu HZ. Resection of multiple rectal carcinoids with transanal endoscopic microsurgery: Case report. World J Gastroenterol 2015; 21:2220-2224. [PMID: 25717261 PMCID: PMC4326163 DOI: 10.3748/wjg.v21.i7.2220] [Citation(s) in RCA: 10] [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: 06/30/2014] [Revised: 09/26/2014] [Accepted: 12/01/2014] [Indexed: 02/06/2023] Open
Abstract
Multiple rectal carcinoids are rare. Due to the unreliability of endoscopic polypectomy in treating these submucosal lesions, a laparotomy is usually performed. We present a case report on multiple rectal carcinoids with three carcinoid foci < 10 mm in diameter located in the mid-rectum. Preoperative examination showed the lesions to be confined to the submucosal layer with no perirectal nodal involvement. A transanal endoscopic microsurgery was successfully performed to remove the three lesions with accurate full-thickness resection followed by secured suture closure. The postoperative pathology revealed neuroendocrine tumors G1 (carcinoids) located within the submucosal layer without lymphatic or vessel infiltration. Both the deep and lateral surgical margins were completely free of tumor cells. The patient recovered quickly and uneventfully. No tumor recurrence was observed at the six-month follow-up. For the multiple small rectal carcinoids without muscularis propria or lymphatic invasion, transanal endoscopic microsurgery offers a reliable and efficient alternative approach to traditional laparotomy for select patients, with the added advantages of minimally invasive surgery.
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Lu C, Qiu Y, Lu X, Li G, Bu H. Synchronous diffuse ganglioneuromatosis and multiple schwannomas of the colon: A case report and literature review. Exp Ther Med 2015; 9:733-736. [PMID: 25667620 PMCID: PMC4316863 DOI: 10.3892/etm.2015.2212] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/07/2014] [Accepted: 01/07/2015] [Indexed: 02/05/2023] Open
Abstract
Diffuse ganglioneuromatosis (DG) of the gastrointestinal tract is a rare condition that is closely associated with neurofibromatosis type 1 and multiple endocrine neoplasia type 2B. The occurrence of DG with multiple schwannomas, which, of the GI tract, usually affect the stomach, is considerably more rare. The present study describes the case of a 54-year-old male with indolent DG, principally involving the small intestine and colon, associated with multiple schwannomas in the subserosa. The patient was treated with surgery. A brief overview of intestinal ganglioneuromatous lesions and the associated conditions is additionally presented.
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Affiliation(s)
- Changli Lu
- Department of Pathology, West China Hospital, Sichuan University, Chengdu, Sichuan 610041, P.R. China
| | - Yan Qiu
- Department of Pathology, West China Hospital, Sichuan University, Chengdu, Sichuan 610041, P.R. China
| | - Xufeng Lu
- Regenerative Medicine Research Center, West China Hospital, Sichuan University, Chengdu, Sichuan 610041, P.R. China
| | - Gandi Li
- Department of Pathology, West China Hospital, Sichuan University, Chengdu, Sichuan 610041, P.R. China
| | - Hong Bu
- Department of Pathology, West China Hospital, Sichuan University, Chengdu, Sichuan 610041, P.R. China
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Toh JWT, Henderson C, Yabe TE, Ong E, Chapuis P, Bokey L. Management of sub-5 mm rectal carcinoids with lymph node metastases. Gastroenterol Rep (Oxf) 2014; 3:350-4. [PMID: 25342710 PMCID: PMC4650972 DOI: 10.1093/gastro/gou073] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/04/2014] [Accepted: 09/16/2014] [Indexed: 02/07/2023] Open
Abstract
Minute (<5 mm) and small (5–10 mm) rectal carcinoids discovered during colonoscopy are generally considered to be non-aggressive, and the management and surveillance of patients with this entity are usually limited. We present the case of a 61-year-old Chinese female with multiple sub-5 mm carcinoid tumours in the rectum without any computed tomography (CT) evidence of lymph node or distant metastases. She underwent an ultra-low anterior resection for a sessile rectal polyp with the histological appearance of a moderately differentiated adenocarcinoma. Seven foci of minute carcinoids in the rectum and perirectal lymph node metastastic spread from the carcinoid tumours were also discovered on histopathology. There were no lymph node metastases originating from adenocarcinoma. This case report and review of the literature suggests that minute rectal carcinoids are at risk of metastasizing and that these patients should be investigated for lymph node and distant metastatic spread with CT and somatostatin receptor scintigraphy or its equivalent, as this would influence prognosis and surgical management of these patients. Findings relating to lymphovascular invasion, perineural invasion, high Ki-67, mitotic rate, depth of tumour invasion, central ulceration, multifocal tumours and size are useful in predicting metastases and may be used in scoring tools. Size alone is not a good predictor of metastastic spread.
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Affiliation(s)
- James Wei Tatt Toh
- Department of General Surgery, Liverpool Hospital, Liverpool, New South Wales (NSW), Australia,
| | | | - Takako Eva Yabe
- Department of General Surgery, Liverpool Hospital, Liverpool, New South Wales (NSW), Australia
| | - Evonne Ong
- South West Clinical School, University of New South Wales, Sydney, NSW, Australia
| | - Pierre Chapuis
- Department of Colorectal Surgery, Concord Hospital, Sydney, NSW, Australia and
| | - Les Bokey
- Department of Colorectal Surgery, Liverpool Hospital, Liverpool, NSW, Australia
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Moghaddasi M, Aghaii M, Mamarabadi M. Perianal pain as a presentation of lumbosacral neurofibroma: a case report. J Neurol Surg Rep 2014; 75:e191-3. [PMID: 25485211 PMCID: PMC4242821 DOI: 10.1055/s-0033-1358793] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/26/2012] [Accepted: 09/01/2013] [Indexed: 11/15/2022] Open
Abstract
Rectal and perianal pain is a common problem. Most people have experienced it at least once in their lifetime. It usually manifests as mild discomfort, but sometimes the pain can be so severe that it is incapacitating. A 59-year-old woman admitted with a 2-year history of paroxysmal perianal pain underwent a full work-up including proctoscopy, sigmoidoscopy, full colonoscopy, and barium enema that were unremarkable. Lumbosacral magnetic resonance imaging with and without gadolinium showed an intradural-extramedullary lesion at the level of L5. The pathologic diagnosis was a neurofibroma. She underwent surgery, and after a few weeks she felt well and medication was no longer needed for her paroxysmal pain. Although one should consider the usual causes of colorectal pain such as hemorrhoids, anal fissure, proctalgia fugax, and chronic perianal pain syndrome, we should keep in mind that some referral pain may mimic local pathologies and should be evaluated properly.
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Affiliation(s)
- Mehdi Moghaddasi
- Department of Neurology, Rasool Akram Hospital, Tehran University of Medical Sciences, Tehran, Iran
| | - Mahboubeh Aghaii
- Department of Neurology, Rasool Akram Hospital, Tehran University of Medical Sciences, Tehran, Iran
| | - Mansoureh Mamarabadi
- Department of Neurology, Rasool Akram Hospital, Tehran University of Medical Sciences, Tehran, Iran
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Abstract
We report a case of neuronal hypertrophy associated with acute appendicitis in which significant neuronal fibers and the number of ganglion cells increased in the absence of inflammatory cells. Differential diagnosis from diffuse ganglioneuromatosis by the pathologic findings of resected specimen was difficult. A 33-year-old Japanese female visited our hospital complaining of acute abdominal pain. The patient underwent appendectomy upon the diagnosis of acute appendicitis on the day of admission. Postoperative examinations found no neoplastic lesions in other organs or inherited disorders such as multiple endocrine neoplasia (MEN) type 2b and von Recklinghausen's disease (VRD). The pathologic diagnosis was neuronal hypertrophy of the appendix. Because the clinical outcomes of diffuse ganglioneuromatosis and neuronal hypertrophy of the gastrointestinal tract are quite different, clinical and pathologic examination should be carefully carried out for lesions in which significant proliferation of neuronal components is seen.
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Ghassemi KA, Ou H, Roth BE. Multiple rectal carcinoids in a patient with neurofibromatosis. Gastrointest Endosc 2010; 71:216-8. [PMID: 19836738 DOI: 10.1016/j.gie.2009.06.026] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/01/2009] [Accepted: 06/23/2009] [Indexed: 02/07/2023]
Affiliation(s)
- Kevin A Ghassemi
- Division of Digestive Diseases, David Geffen School of Medicine at UCLA, Los Angeles, California 90095, USA
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Park JY, Kang YN, Park KS, Choe M. Solitary Polypoid Ganglioneuroma of the Cecum. JOURNAL OF THE KOREAN SOCIETY OF COLOPROCTOLOGY 2009. [DOI: 10.3393/jksc.2009.25.4.264] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 10/20/2022]
Affiliation(s)
- Ji young Park
- Department of Pathology, Keimyung University School of Medicine, Daegu, Korea
| | - Yu Na Kang
- Department of Pathology, Keimyung University School of Medicine, Daegu, Korea
| | - Kyung Sik Park
- Department of Internal Medicine, Keimyung University School of Medicine, Daegu, Korea
| | - Misun Choe
- Department of Pathology, Keimyung University School of Medicine, Daegu, Korea
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Landry CS, Brock G, Scoggins CR, McMasters KM, Martin RCG. A proposed staging system for rectal carcinoid tumors based on an analysis of 4701 patients. Surgery 2008; 144:460-6. [PMID: 18707046 DOI: 10.1016/j.surg.2008.05.005] [Citation(s) in RCA: 53] [Impact Index Per Article: 3.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/03/2008] [Accepted: 05/22/2008] [Indexed: 12/20/2022]
Abstract
BACKGROUND The lack of a clinically relevant staging system for carcinoid tumors of the rectum creates a persistent challenge for clinicians trying to provide patients with meaningful prognostic information. The purpose of this study was to identify the clinicopathologic factors that affect survival for patients diagnosed with carcinoid of the rectum, and to use this information to create a staging system. METHODS A search of 15,983 patients with carcinoid tumors from the National Cancer Institute's SEER (Surveillance Epidemiology and End Results) database identified 4701 patients with rectal carcinoid tumors from 1973 to 2004. Patients were analyzed according to various clinicopathologic factors and a tumor (T1, T2, T3), lymph node (N0, N1), and metastasis (M0, M1) staging system was created according to these parameters. The staging system was developed using log rank tests and the Cox proportional hazards model. RESULTS Of the 4701 patients, 2329 females and 2372 males were identified with a median age of 56 years (14-94). Median size of primary tumor was 0.6 cm (0.1-25). Lymph node metastasis was found in 111 (4%), and distant metastatic disease was present in 97 (2.4%) patients. Rectal carcinoid was the only primary malignancy in 82% of patients; 17% presented with 1 additional primary malignancy, and 1% had two or more additional malignancies. Age, size, depth of invasion, lymph node involvement, and distant metastasis were significant predictors of survival. Four stages were created according to statistically significant prognostic factors: 83% of patients were classified into Stage I, 6.5% into Stage II, 2.8% into Stage III, and 7.4% into Stage IV. Five-year survival rates were 97%, 84%, 27%, and 20% for Stages I through IV, respectively. CONCLUSION The newly developed TNM staging system accurately discriminates prognosis for carcinoid tumors of the rectum. Size of primary tumor, depth of invasion, lymph node involvement, and distant metastasis have the greatest impact on survival. Incorporation of this staging system into clinical practice will allow better study of outcomes and development of stage-specific treatment recommendations.
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Affiliation(s)
- Christine S Landry
- Division of Surgical Oncology, Department of Surgery and James Graham Brown Cancer Center, University of Louisville School of Medicine, Louisville, KY, USA
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