MEN1 syndrome and neuroendocrine tumor of unknown primary

#3286

Introduction: Multiple Endocrine Neoplasia type 1 (MEN1) is a hereditary condition associated with tumors of endocrine glands. NeuroEndocrine Neoplasms (NENs) are characterized as rare neoplasms.

Aim(s): 42-year-old male with known MEN1 presented with masses in anterior mesothorax and in inferior cervical region. Lesions were identified during work-up followed a pancreatic surgery.

Materials and methods: Particularly, patient was diagnosed with primary hyperparathyroidism and nephrolithiasis 20 years ago and underwent sequential excision of three parathyroid glands. Due to family history of primary hyperparathyroidism further work-up revealed the MEN1 syndrome. Molecular diagnoses detected the presence of p.A541T mutation. Radiological examinations depicted two large pancreatic masses. Segmental pancreatectomy, splenectomy and cholecystectomy took place. Histology revealed a well-differentiated NET, grade 2 (NET G2 WHO, 2MF/10HPF, Ki-67=5%), chromogranin, synaptophysin and CD56 positive. Additionally, MRI scan of pituitary gland revealed a small adenoma 2mm, stable in size without secretion of hormones.

Conference: 18th Annual ENETS Concerence (2021)

Presenting Author: Tsoukalas N

Authors: Tsoukalas N, Sakellaridis T, Kyriakopoulos G, Arvanitou E, Tsitsimpis A,

Keywords: neuroendocrine neoplasm, multiple endocrine neoplasia type 1 (MEN1), unknown primary, atypical carcinoid of thymus,