Endocrine Abstracts

Introduction: Multiple endocrine neoplasia type 1 syndrome (MEN1sy.) is a complex disease predisposing to a variety of multifocal neuroendocrine tumors (NET). Approximately 5% of pancreatic VIPomas and glucagonomas are associated with MEN1sy., most of them with metastases at diagnosis.

Case report: A 15-year-old boy was admitted with primary hyperparathyroidism, pituitary microadenoma (6 mm) and suspected MEN1sy. His father had primary hyperparathyroidism and pancreatic gastrinoma. While hospitalized, the patient had intense abdominal pain, facial flushing and massive diarrhea, severe volume depletion, hyperchloremic hypokalemic acidosis and aggravated hypercalcaemia. Endoscopic ultrasound (EUS) and MRI showed two tumors localised in the pancreatic head and tail. Octreoscan revealed one lesion in the pancreatic head. Gastrin and 5-HIAA values were in normal range. Chromogranin A (CgA) was increased (562 ng/l). Direct sequencing of MEN1 gene identified germline mutation at codon 395 of exon 8 (CAG→TAG). Somatostatin analogue therapy was started before surgery. Near total parathyroidectomy with profilactic thymectomy was performed. Parathyroid glands showed diffuse hyperplasia. Pancreatic resection with total tumor exscision was also performed. Histopathological analysis revealed four well-differentiated NETs, three of them showed VIP-ma imunoprofile, but one was pure glucagonoma (Ki67-1%, MI 0/50 HPF, CgA+++, VIP+++, glucagon++). Peritumorous pancreatic tissue showed microadenomatous proliferation. There were no regional lymphonodal metastases. Postoperatively the patient remained asymptomatic with persistant hypoparathyroidism and normal CgA values. After 18 months, MRI and EUS showed one lesion in the pancreatic tail, of the same dimensions (10 mm) as preoperatively, without other dissemination. Patient also had microprolactinoma. Prior and after pancreatic surgery (independently of VIP stimulation) serum PRL levels were up to 3000 mIU/l, without PRL response to TRH stimulation.

Conclusions: We present a case with multicentric pancreatic VIPomas and one glucagonoma in association with MEN1sy. The persistant solitary lesion in the pancreatic tail can be either clinically non-functional NET or microadenomatosis associated with MEN1sy.

Declaration of interest: The authors declare that there is no conflict of interest that could be perceived as prejudicing the impartiality of the research project.

Funding: This research did not receive any specific grant from any funding agency in the public, commercial or not-for-profit sector.