Adrenocortical carcinoma in the context of MEN1 patients with pNETS: From suspicion to diagnosis, management and follow-up

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Introduction: Adrenocortical carcinoma (ACC) is a rare malignancy arising from adrenal parenchymal cells. Multiple endocrine neoplasia type 1 is an autosomal inherited predisposition cancer syndrome characterized by parathyroid hyperplasia, anterior pituitary gland tumors and pancreatic islet tumors. Although adrenal involvement in MEN1 patients has been reported in about 40% of patients, the incidence of ACC has been reported to range between 1,4% and 6% in MEN1.

Aim(s): This manuscript aimed to review data on the incidence of ACC in MEN1 patients presenting with pNETs aiming at increasing awareness.

Materials and methods: Electronic databases were searched with the appropriate search terms for the period up to and including November 2020.

Conference: 18th Annual ENETS Concerence (2021)

Presenting Author:

Authors: Yiannakopoulou E,

Keywords: MEN1, pNETs, adrenocortical carcinoma,