Clinical manifestation and aggressiveness of duodenopancreatic neuroendocrine tumors (DP-NET) in patients with MEN1 syndrome: A possible role of exon 2 mutations in menin gene

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Introduction: Multiple endocrine neoplasia type 1 (MEN1) is a rare syndrome characterized by co-occurrence of primary hyperparathyroidism (PH), duodenopancreatic neuroendocrine tumors (DP-NET) and/or pituitary adenomas without an established genotype-phenotype correlation.

Aim(s): To compare median age at diagnosis (TD) and aggressiveness of MEN-1 syndrome clinical manifestations in patients with exon 2 mutation (Ex2) with patients with other mutations (OtherEx).

Materials and methods: Medical charts of MEN-1 patients diagnosed between 1992 and 2022 referred to ENETS CoE Federico II University of Naples were retrospectively evaluated. Recist 1.1 criteria were used to define DP-NET progression.

Conference:

Presenting Author: Benevento E

Authors: Benevento E, Liccardi A, Minotta R, Cannavale G, Di Iasi G,

Keywords: Multiple endocrine neoplasia type 1 (MEN1), exon two, genomic, primary hyperparathyroidism, duodenopancreatic neuroendocrine tumor, DP-NET, pituitary adenomas,