Small cell carcinoma arising in a pancreatic neuroendocrine tumour (PNET) in a patient with family history of multiple endocrine neoplasia type 1 (MEN1): a case report

• Published in: Pathology Vol. 47; p. S61
• Main Authors: Daneshvar, S., Williams, D.
• Format: Journal Article
• Language: English
• Published: Elsevier B.V 2015
• ISSN: 0031-3025; 1465-3931
• DOI: 10.1097/01.PAT.0000461477.12548.2e

A 47-year-old woman with family history of MEN1 underwent an abdominal CT scan to reveal a 20mm cystic mass in the distal pancreas and an enlarged coeliac lymph node. FNA of lymph node showed features of a neuroendocrine tumour (NET). Distal pancreato-splenectomy and regional lymphadenectomy was performed and histology showed pancreatic neuroendocrine tumour with progression into small cell carcinoma in an involved lymph node. There were also two separate pancreatic microadenomas and background islet cell hyperplasia favouring underlying MEN1. Poorly differentiated PNETs are rare. Neoplastic progression of well differentiated NETs has been proposed as the origin of some poorly differentiated pancreatic neuroendocrine carcinomas.1,2 Although the frequency with which pancreatic well differentiated (WHO grade 1 and 2) NETs may transform to true poorly differentiated neuroendocrine carcinomas is unknown, most observation to date suggest this is an uncommon pathway.1 This is the first report of PNET with progression into small cell carcinoma in MEN1 patient.