Navigating the uncommon: a comprehensive case study of multiple gastroduodenal neuroendocrine tumor
DOI:
https://doi.org/10.18203/2320-6012.ijrms20241929Keywords:
Neuroendocrine tumors, Duodenal NET, Duodenum, GIST, Small intestine malignancyAbstract
Neuroendocrine tumors (NETs) have varied pathophysiological characteristics, location, clinical presentation, management & outcome. Unfortunately, most NETs are non-functional and therefore, either remain asymptomatic until incidentally detected or present very late with pressure symptoms, adding up to the associated morbidity and mortality. Here we presented a case of a 43-year gentleman, who presented to gastroenterology OPD with chief complaints of heartburn and pain in the upper abdomen for one year. He had an equivocal clinical examination and laboratory parameters. Upper GI endoscopy and computed tomography revealed multiple nodular growths in the D1 segment and pylorus of the stomach. He was successfully managed by wide local excision of D1 and distal stomach (pylorus) followed by Polya gastrojejunostomy reconstruction. Histopathology confirmed well-differentiated NETs, low Ki67, and positive for chromogranin and synaptophysin. This case draws attention to the early age of presentation of multifocal NETs with vague symptoms and equivocal clinical examination. Coexisting gastric NETs with duodenal NETs in itself is very rare, never the less non-functional status and pre-operative diagnostic dilemma. Here, we have also drawn attention to the pros and cons of various diagnostic tools and how their utility can sometimes limit the approach of clinicians, apart from a high index of suspicion.
Metrics
References
Das S, Dasari A. Epidemiology, incidence, and prevalence of neuroendocrine neoplasms: are there global differences? Curr Oncol Rep. 2021;14:43-10.
Palepu J, Shrikhande SV, Bhaduri D, Shah RC, Sirohi B, Chhabra V, et al. Trends in diagnosis of gastroenteropancreatic neuroendocrine tumors (GEP-NETs) in India: A report of multicenter data from a web-based registry. Indian J Gastroenterol. 2017;36(6):445-51.
Carvão J, Dinis-Ribeiro M, Pimentel-Nunes P, Libânio D. Neuroendocrine tumors of the gastrointestinal tract: a focused review and practical approach for gastroenterologists. GE Port J Gastroenterol. 2021;28(5):336-48.
Soga J. Endocrinocarcinomas (carcinoids and their variants) of the duodenum. An evaluation of 927 cases. J Exp Clin Cancer Res. 2003;22:349-63.
Maxwell JE, Howe JR. Imaging in neuroendocrine tumors: an update for the clinician. Int J Endocr Oncol. 2015:159-68.
Rindi G, Mete O, Uccella S, Basturk O, Rosa SL, Brosens LAA, et al. Overview of the 2022 WHO classification of neuroendocrine neoplasms. Endocr Pathol. 2022;33(1):115-54.
Gut P, Czarnywojtek A, Fischbach J, Bączyk M, Ziemnicka K, Wrotkowska E, et al. Chromogranin A-unspecific neuroendocrine marker. Clinical utility and potential diagnostic pitfalls. Arch Med Sci. 2016;12(1):1-9.
Sok C, Ajay PS, Tsagkalidis V, Kooby DA, Shah MM. Management of gastric neuroendocrine tumors: a review. Ann Surg Oncol. 2024;31(3):1509-18.
Sorbye H, Grande E, Pavel M. European Neuroendocrine Tumor Society (ENETS) 2023 guidance paper for digestive neuroendocrine carcinoma. J Neuroendocrinol. 2023;35(3):13249.