A rare presentation of an ACTH-producing high-grade large cell neuroendocrine carcinoma with Cushing's syndrome.

High-grade neuroendocrine tumors (HGNET) are rare neoplasms composed of neural and hormonal with only around 42 cases reported in the last 20 years1. Herein, we describe a rare case of pancreatic HGNET, large cell type, associated with a Cushing's syndrome presentation.