Association of MINEN with GIST on neurofibromatosis – A case report

#3748

Introduction: Although most digestive neuroendocrine tumors (NETs) are sporadic, around 5% of NETs occur in a context of hereditary predisposition, mainly type 1 multiple endocrine neoplasia (NEM1) and Von Hippel-Lindau disease (VHL).

Aim(s): We report the case of a MINEN or mixed neuroendocrine non-endocrine ampullary neoplasm associated with neurofibromatosis type1 (NF1) which is itself linked to the appearance of GIST or Gastro Intestinal Stromal Tumor in our case of duodenal GIST.

Materials and methods: Clinical case: This is a 55-year-old AD patient with neurofibromatosis type 1 and thyroid carcinoma ATCD who underwent ampullectomy for ampullary adenocarcinoma classified as Pt2N0MX (pTNM2017), but whose resection was R1 on the bile duct border. 2 months later the patient underwent a cephalic duodenopancreatectomy.

Conference:

Presenting Author:

Authors: Samia K, Fatima H, Nabil B, Benali T, Aisha B,

Keywords: NEN, GIST, MINEN, DOG1,