Two Cases of von Hippel-Lindau Syndrome Manifesting as Bilateral Adrenal Pheochromocytomas
LI Jiajun
HE Yichen
XIE Libo
Abstract：Von Hippel-Lindau(VHL)syndrome is a rare autosomal dominant genetic disorder.Adolescent patients with bilateral adrenal pheochromocytoma should be alert to the possibility of VHL syndrome,and genetic testing is the key to diagnosis.Radical surgical resection is the main treatment,and lifelong follow-up is required after surgery to monitor the oc-currence of multi-organ tumors.This study reports the clinical data,imaging features,pathological and genetic testing results of 2 adolescent VHL syndrome patients presenting with bilateral adrenal pheochromocytoma,and summarizes their diagno-sis,treatment process and follow-up results,aiming to provide a more accurate treatment strategy for this disease.
Keywords：Von Hippel-Lindau syndromepheochromocytomaadrenal gland neoplasmsadolescentgene muta-tion
Publication Date：2025-10-28
Online Publishing Date：2026-01-15(First online date of this platform, not the publication date of the document)
Pages：5( 343-347 )