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Sporadic, Non‐Functional , Gastrin‐Producing Duodenal Neuroendocrine Tumors: A Retrospective Study of an Infrequent Disease

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Jorge Huerta, Lucía de and Solares Fernández, Isabel and Sánchez Moreno, Beatriz and Males Maldonado, David and Ibarrola De Andrés, Carolina and Díaz Simón, Raquel (2022) Sporadic, Non‐Functional , Gastrin‐Producing Duodenal Neuroendocrine Tumors: A Retrospective Study of an Infrequent Disease. Journal of Digestive Diseases . ISSN 1751-2972

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Official URL: https://doi.org/10.1111/1751-2980.13129



Abstract

Objective
non-functioning gastrin-producing Neuroendocrine Neoplasms (NEN) of the duodenum are rare tumors of the gastrointestinal tract without a clinical syndrome due to gastrin-production. Their incidence has significantly increased in the last few years especially as an incidental finding during endoscopic studies. The aim of this study is to describe the characteristics of this emergent neoplasm, to provide more information on this rare pathology and its possible prognostic factors.

Methods
we performed a retrospective observational study based on the duodenal-NENs samples with positive staining for gastrin, registered in the Pathology Department of University Hospital 12-de-Octubre (Madrid, Spain) between 2000 and 2017. We excluded all those clinically functional [(Zollinger-Ellison-Syndrome) and/or gastrinemia >1000pg/ml], with a previous diagnosis of multiple endocrine neoplasia(MEN-syndrome) or a synchronous neoplasia. Clinicopathological and therapeutic variables, follow-up time, recurrence and mortality data were collected.

Results
21 patients were included. Most of the tumors were diagnosed incidentally as a single small polypoid lesion, they were limited to mucosa/submucosa and had a low histological-grade. 4 patients (19%) presented metastatic involvement at diagnosis (lymphatic and/or hepatic). These 4 patients also had a high or intermediate mitotic grade and infiltration further than submucosa. Local resection was used in most cases as curative treatment. The median follow-up was 25 months. There were 2 relapses and 2 deaths attributed to the tumor with a 5-year disease-free-survival of 81%.

Conclusions
The majority of these tumors were diagnosed at a local stage and had a good prognosis with local treatment. Nevertheless, given the potential metastatic risk, a close follow-up and extensive study is necessary, especially in those with aggressive pathological factors such as deep infiltration or high-histological-grade.


Item Type:Article
Additional Information:

CRUE-CSIC (Acuerdos Transformativos 2022)

Uncontrolled Keywords:Neuroendocrine neoplasm, Duodenum, Non-functioning tumor, Gastrin staining, Duodenal neuroendocrine tumor
Subjects:Medical sciences > Medicine > Gastroenterology and Hepatology
ID Code:75439
Deposited On:08 Nov 2022 12:41
Last Modified:21 Nov 2022 10:59

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