Keywords:
Phaeochromocytoma, von Hippel Lindau disease, Recurrence.
Introduction
Von Hippel-Lindau (VHL) syndrome is a VHL-gene-mutation-related disease with autosomal dominant inheritance. The incidence rate reported abroad is about 1:36000 in the general population.1 It frequently presents as benign and malignant multiorgan tumors, involving brain, spinal cord, retina, pancreas, kidneys, adrenal glands, epididymis and other structures. Among VHL patients, phaeochromocytomas are diagnosed at the average age of 30 years old, with a metastatic risk of approximately 5%.2 The primary lesions usually locate in bilateral adrenal glands, and extra-adrenal paraganglioma could occur as the disease progresses. Due to rareness of phaeochromocytoma and paraganglioma (PPGL), clinicians often lack experience to deal with relative cases. Here, we report a case of VHL-related recurrent phaeochromocytoma with multiorgan involvement and review the literature.