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2026, Number 2

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Cir Gen 2026; 48 (2)

Pancreatic neuroendocrine tumor in MEN1 syndrome: surgical, radiological, and pathological findings

Franco GCD, Fierro DJ, Cuevas SMT, Gándara AC, Candanedo GF, Domínguez RI
Full text How to cite this article 10.35366/123465

DOI

DOI: 10.35366/123465
URL: https://dx.doi.org/10.35366/123465

Language: Spanish
References: 20
Page: 100-105
PDF size: 2558.37 Kb.


Key words:

multiple endocrine neoplasia type 1, pancreatic neuroendocrine tumors, positronemission tomography, pancreatectomy.

ABSTRACT

Multiple endocrine neoplasia type 1 is an autosomal dominant hereditary syndrome that leads to the development of endocrine tumors in glands such as the parathyroids, pancreas, and pituitary. Pancreatic neuroendocrine tumors, which are common in this condition, may be functional or nonfunctional and pose diagnostic and therapeutic challenges due to their multifocal and heterogeneous behavior. Management requires an individualized, multidisciplinary approach. We report the case of a 32-year-old woman with multiple endocrine neoplasia type 1 and multiple pancreatic lesions detected incidentally. Imaging studies demonstrated three hypervascular pancreatic tumors with intense somatostatin receptor uptake and variable metabolic activity. The patient underwent distal pancreatectomy and splenectomy. Histopathological analysis confirmed well-differentiated neuroendocrine tumors without lymph node involvement. This case highlights the value of multimodal imaging and multidisciplinary collaboration for the diagnosis, treatment, and follow-up of pancreatic neuroendocrine tumors in patients with multiple endocrine neoplasia type 1.


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Cir Gen. 2026;48