[A Case of Von Hippel-Lindau Disease with Nonfunctioning Pancreatic Neuroendocrine Tumors Treated by Duodenum-Preserving Resection of the Head of the Pancreas and Spleen-Preserving Resection of the Tail of the Pancreas].
A 26-year-old woman presented to our department with a diagnosis of multiple nonfunctioning pancreatic neuroendocrine tumors. She had a family history of pheochromocytoma and a medical history of bilateral adrenalectomy for pheochromocytoma at the age of 25 years. During follow-up treatment for adrenal insufficiency after the surgery, highly enhanced tumors in the pancreas were detected on contrast-enhanced CT. Other examinations found that the patient did not satisfy the clinical criteria for von Hippel-Lindau (VHL) disease. Considering her age and risk of developing multiple heterotopic and heterochronous tumors, we performed a duodenum-preserving resection of the head of the pancreas and spleen-preserving resection of the tail of the pancreas with informed consent. The histopathological findings revealed that all of the tumors were NET G1. She underwent genetic testing postoperatively and was diagnosed with VHL disease. This diagnosis meant that we were able to create an optimal treatment plan for the patient. If a tumor predisposition syndrome is suspected, VHL disease should be borne in mind and genetic testing after genetic counseling should be duly considered.
Dept. of Surgery, Aomori Prefectural Central Hospital.
Yutaka
Y
Umehara
Minoru
M
Umehara
Tomohisa
T
Tokura
Takafumi
T
Yachi
Kenichi
K
Takahashi
Takayuki
T
Morita
Kenichi
K
Hakamada
D000310:Adrenal Gland Neoplasms / Q000601:surgery; D000315:Adrenalectomy; D000328:Adult; D013505:Digestive System Surgical Procedures; D004386:Duodenum / Q000601:surgery*; D005260:Female; D006801:Humans; D010179:Pancreas / Q000601:surgery*; D010190:Pancreatic Neoplasms / Q000209:etiology / Q000601:surgery*; D013154:Spleen / Q000601:surgery*; D006623:von Hippel-Lindau Disease / Q000150:complications*