A case report of ⅡC type VHL syndrome
10.3760/cma.j.cn112330-20210201-00066
- VernacularTitle:ⅡC型希佩尔·林道综合征一例报告
- Author:
Chengren GOU
1
;
Zongping CHEN
;
Tong LIU
;
Zidong ZHOU
;
Kaiyi MAO
Author Information
1. 遵义医科大学附属医院泌尿外科，遵义 563000
- Keywords:
Adrenal gland neoplasms;
Von Hippel-Lindau syndrome;
Adrenal pheochromocytoma;
Pancreatic neuroendocrine tumors;
Genetic diagnosis;
Treatment
- From:
Chinese Journal of Urology
2021;42(12):939-941
- CountryChina
- Language:Chinese
-
Abstract:
On March 24, 2017, a patient with Von Hippel-Lindau syndrome (VHL) characterized by bilateral adrenal pheochromocytoma and pancreatic tumors was admitted to our hospital, who underwent simultaneous pancreatic body and tail tumor resection, bilateral adrenal tumor resection and Omentum transplantation of the right adrenal gland.Intraoperative hormone therapy was used. Part of the normal adrenal tissue was preserved and embedded in the omentum, but an adrenal crisis occurred on the first day after the operation.The hormone replacement was used. Postoperative hormone replacement therapy was performed for 6 months. After 4 years of follow-up, blood pressure was normal, no cortical dysfunction, no tumor recurrence or other related lesions appeared. The preserved part of adrenal tissue during simultaneous multi-organ tumor resection for such patients can reduce long-term hormone replacement after surgery and prevent late adrenal cortex dysfunction.
