A rare case of pancreatic neuroendocrine neoplasm causing Cushing's syndrome.

Authors :: Nakaoka K
Hashimoto S
Kawabe N
Kuzuya T
Yamada S
Sawaki A
Funasaka K
Nagasaka M
Nakagawa Y
Miyahara R
Shibata T
Tsukamoto T
Hirooka Y
Source :: Clinical journal of gastroenterology [Clin J Gastroenterol] 2022 Feb; Vol. 15 (1), pp. 256-262. Date of Electronic Publication: 2022 Jan 21.
Publication Year :: 2022
Abstract: Pancreatic neuroendocrine neoplasms (PNENs) are relatively rare with a reported incidence of 1-2/100,000 and generally thought to originate from the precursor of the neuroendocrine cells including the islet and the pancreatic duct cells. About 65% of PNENs are non-functional. While insulinomas and gastrinomas are the most common functional PNENs, ACTH-producing PNENs are extremely rare. We herein present an extremely rare case of a patient with Cushing's syndrome caused by PNEN. A 46-year-old woman with edema in bilateral lower extremities and moon face was admitted with a suspicious pancreatic tumor. Enhanced computed tomography and endoscopic ultrasonography revealed a pancreatic tumor. The final diagnosis of ACTH-producing PNEN with Cushing's syndrome was based on clinical and biochemical test results and endocrinological studies. The symptoms associated Cushing's syndrome improved after pancreaticoduodenectomy for PNEN.<br /> (© 2022. Japanese Society of Gastroenterology.)