Abstract
Gastric neuroendocrine carcinomas (GNECs) are rare tumors characterized by an aggressive clinical course, rapid progression, metastatic dissemination, and poor prognosis. Due to overlapping radiological features and small pre-operative biopsy specimens, they are frequently misdiagnosed as adenocarcinoma in the pre-operative period. These tumors are generally asymptomatic or present with non-specific symptoms like epigastric pain or discomfort, weight loss, and loss of appetite. However, GNECs presenting as perforation peritonitis are rarely reported in the literature. A 60-year-old man presented to the emergency department with complaints of pain upper abdomen with loss of weight and appetite for four months. He had an endoscopic biopsy report suggestive of antral growth reported as poorly differentiated adenocarcinoma. Radiological investigations were notable for the presence of a malignant lesion in the stomach with perforation. Subtotal gastrectomy with D1 lymph node resection was done. Specimen pathology confirmed poorly differentiated high grade large cell neuroendocrine carcinoma. This article emphasizes the importance of considering GNEC as a differential diagnosis for gastric adenocarcinoma despite overlapping radiological features and challenges with pre-operative diagnosis due to its aggressive nature and to allow for appropriate management.
Similar content being viewed by others
Data Availability
The data used to support the findings of this study are available from the corresponding author upon request. We will disclose all information except personal identifiable information.
Code Availability
Not applicable.
References
Dasari A, Mehta K, Byers LA, Sorbye H, Yao JC. Comparative study of lung and extrapulmonary poorly differentiated neuroendocrine carcinomas: A SEER database analysis of 162,983 cases. Cancer. 2018;124:807–15. https://doi.org/10.1002/cncr.31124.
National Comprehensive Cancer Network. Neuroendocrine and adrenal tumors (Version 2.2021) [Internet]. [cited 2021 Jun 29]. Available from: https://www.nccn.org/professionals/physician_gls/pdf/neuroendocrine.pdf.
Rajendran T, Katta B, Shaikh OH, Kumbhar US. Small cell carcinoma of the pancreas: A rare neuroendocrine tumour. BMJ Case Rep. 2020;13:e235397. https://doi.org/10.1136/bcr-2020-235397.
Jiang S-X, Mikami T, Umezawa A, Saegusa M, Kameya T, Okayasu I. Gastric large cell neuroendocrine carcinomas: A distinct clinicopathologic entity. Am J Surg Pathol. 2006;30:945–53. https://doi.org/10.1097/00000478-200608000-00003.
Kang SH, Kim KH, Seo SH, An MS, Ha TK, Park HK, et al. Neuroendocrine carcinoma of the stomach: A case report. World J Gastrointest Surg. 2014;6:77–9. https://doi.org/10.4240/wjgs.v6.i4.77.
Song L, Jin Z, Zhang W, Zhang Y. Gastric large cell neuroendocrine carcinoma with venous tumor thrombus: The value of PET/CT and contrast-enhanced computed tomography. Clin Imaging. 2015;39:325–8. https://doi.org/10.1016/j.clinimag.2014.09.002.
Klimstra D, Klöppel G, La Rosa S, Rindi G. Classification of neuroendocrine neoplasms of the digestive system. WHO Classification of tumours, 5th Edition Digestive system tumours. IARC Press; 2019. p. 16–9. https://doi.org/10.1111/his.13975.
La Rosa S, Sessa F, Uccella S. Mixed neuroendocrine-nonneuroendocrine neoplasms (MiNENs): Unifying the concept of a heterogeneous group of neoplasms. Endocr Pathol. 2016;27:284–311. https://doi.org/10.1007/s12022-016-9432-9.
Kim KA, Shin Park Y, Lee J, Woong Choi J, Hee Lee C, Min PC. Neuroendocrine carcinomas of the stomach: Computed tomography and pathologic findings in 32 patients. Iran J Radiol [Internet] Kowsar. 2017; [cited 2021 Jun 29];14. Available from: https://sites.kowsarpub.com/iranjradiol/articles/56273.html#abstract.
Liang P, Wang Y-X, Ren X-C, Gao J-B. Neuroendocrine carcinoma of the stomach: Clinical features and CT findings. Abdom Radiol (NY). 2016;41:19–24. https://doi.org/10.1007/s00261-015-0593-7.
Walter T, Tougeron D, Baudin E, Le Malicot K, Lecomte T, Malka D, et al. Poorly differentiated gastro-entero-pancreatic neuroendocrine carcinomas: Are they really heterogeneous? Insights from the FFCD-GTE national cohort. Eur J Cancer. 2017;79:158–65. https://doi.org/10.1016/j.ejca.2017.04.009.
Korse CM, Taal BG, van Velthuysen M-LF, Visser O. Incidence and survival of neuroendocrine tumours in the Netherlands according to histological grade: Experience of two decades of cancer registry. Eur J Cancer. 2013;49:1975–83. https://doi.org/10.1016/j.ejca.2012.12.022.
Di Buono G, Bonventre G, Badalamenti G, Buscemi S, Romano G, Agrusa A. Duodenal perforation as presentation of gastric neuroendocrine tumour: A case report. Int J Surg Case Rep. 2020;77:S105–8. https://doi.org/10.1016/j.ijscr.2020.09.120.
Gopalakrishnan G, Srinivas BH, Pottakkat B, Gnanasekaran S, Kalayarasan R. Perforated high-grade mixed neuroendocrine nonneuroendocrine neoplasm of cecum: Unusual presentation of rare disease. Gastrointest Tumors. 2021;8:128–33. https://doi.org/10.1159/000512237.
Lee JE, Hong SH, Jung HI, Son MW, Ahn TS, Han SW, et al. Small-cell neuroendocrine carcinoma of the ileum: Case report and literature review. BMC Surg. 2019;19:135. https://doi.org/10.1186/s12893-019-0591-8.
Laishram S, Ravishankar S, Babu S. A case of neuroendocrine carcinoma of the stomach with perforation. Res J Pharm Biol Chem Sci. 2016;7(2):593–8.
Domurat M, Juchimiuk M, Kędra B. Neuroendocrine carcinoma responsible for gastrointestinal tract perforation. Comp Prof Pedagog. 2010;10:577–80.
Tak DH, Moon HS, Jeong HY, Seong JK, Kang SH, Park DH, et al. A case of gastric neuroendocrine carcinoma presenting as panperitonitis by stomach perforation. Korean J Helicobacter Up Gastrointest Res. 2013;13:64–8. https://doi.org/10.7704/kjhugr.2013.13.1.64.
Kunz PL, Reidy-Lagunes D, Anthony LB, Bertino EM, Brendtro K, Chan JA, et al. North American Neuroendocrine Tumor Society. Consensus guidelines for the management and treatment of neuroendocrine tumors. Pancreas. 2013;42(4):557–77. https://doi.org/10.1097/MPA.0b013e31828e34a4.
Binderup T, Knigge U, Loft A, Federspiel B, Kjaer A. 18F-fluorodeoxyglucose positron emission tomography predicts survival of patients with neuroendocrine tumors. Clin Cancer Res. 2010;16:978–85. https://doi.org/10.1158/1078-0432.CCR-09-1759.
Strosberg JR, Coppola D, Klimstra DS, Phan AT, Kulke MH, Wiseman GA, et al. The NANETS consensus guidelines for the diagnosis and management of poorly differentiated (high-grade) extrapulmonary neuroendocrine carcinomas. Pancreas. 2010;39:799–800. https://doi.org/10.1097/MPA.0b013e3181ebb56f.
La Rosa S, Sessa F. High-grade poorly differentiated neuroendocrine carcinomas of the gastroenteropancreatic system: From morphology to proliferation and back. Endocr Pathol. 2014;25:193–8. https://doi.org/10.1007/s12022-014-9316-9.
Ishida M, Sekine S, Fukagawa T, Ohashi M, Morita S, Taniguchi H, et al. Neuroendocrine carcinoma of the stomach: Morphologic and immunohistochemical characteristics and prognosis. Am J Surg Pathol. 2013;37:949–59. https://doi.org/10.1097/PAS.0b013e31828ff59d.
Author information
Authors and Affiliations
Contributions
Draft preparation: Ramnath Aishwarya, Ankit Jain, and Souradeep Dutta. Data collection and analysis: Ramnath Aishwarya, Ankit Jain, and Pampa Ch Toi. Review and editing: Ankit Jain, Souradeep Dutta, Reddy Abhinaya, and Vishnu Prasad Nelamangala Ramakrishnaiah. Supervision: Vishnu Prasad Nelamangala Ramakrishnaiah. All authors contributed to the conceptualization and design of the case report. All authors have read and approved the manuscript.
Corresponding author
Ethics declarations
Ethics Approval
The need for approval was waived for a single case report.
Consent to Participate
Written informed consent was obtained from the patient for participation in this case report study.
Consent for Publication
Written informed consent was obtained from the patient for publication of this report and the accompanying images, and the patient’s anonymity was upheld.
Conflict of Interest
The authors declare no competing interests.
Additional information
Publisher’s Note
Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.
This article is part of the Topical Collection on Surgery
Rights and permissions
Springer Nature or its licensor (e.g. a society or other partner) holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law.
About this article
Cite this article
Aishwarya, R., Jain, A., Dutta, S. et al. Gastric Large-Cell Neuroendocrine Carcinoma Presenting as Perforation Peritonitis: a Rare Case Report. SN Compr. Clin. Med. 5, 141 (2023). https://doi.org/10.1007/s42399-023-01483-3
Accepted:
Published:
DOI: https://doi.org/10.1007/s42399-023-01483-3