Pancreatic Adenocarcinoma in a Patient With Multiple Endocrine Neoplasia 1 Syndrome

Abstract

To the Editor We present the rare occurrence of a concurrent pancreatic neuroendocrine tumor (pNET) and pancreatic ductal adenocarcinoma (PDAC) in a patient with multiple endocrine neoplasia 1 (MEN1) syndrome. It is important for clinicians to consider the possibility of PDAC in patients with MEN1 because aggressive early surgical intervention provides the only chance of cure. Multiple endocrine neoplasia 1 is characterized by parathyroid adenomas, pNETs, and anterior pituitary tumors.1 Multiple endocrine neoplasia 1–associated pNETs are usually slowly progressive and associated with low malignant potential. In the context of MEN1, pancreatic NET size correlates with prognosis and the presence of metastases,2 and lesions of more than 2 cm are associated with greater genetic instability and malignant behaviour.3 The European Neuroendocrine Tumour Society recommendations for management of pancreatic lesions in a patient with MEN1 state that early diagnosis and surgical excision of MEN1-related pNET improve survival, preventing or delaying the development of distant metastases.4 It is mandatory to operate on MEN1-related nonfunctioning pancreatic tumors with metastases, size of more than 2 cm, or yearly increased size of more than 0.5 cm. Management of pNETs of less than 2 cm is controversial, current recommendation being intensive surveillance to avoid repeated intervention where lesions are typically multiple and behave in an indolent fashion.