Synchronous giant bilateral renal tumors as initial presentation of Von Hippel-Lindau disease: Sequential surgical management and transition to renal replacement therapy

Abstract

Synchronous bilateral renal cell carcinoma (RCC) is rare and often linked to hereditary syndromes such as Von Hippel-Lindau (VHL) disease. We present a 27-year-old male with acute appendicitis, whose CT incidentally revealed bilateral renal tumors and pancreatic cysts. Further evaluation identified cerebellar and retinal hemangioblastomas, confirming VHL. Due to tumor complexity, sequential laparoscopic radical nephrectomies were performed, followed by hemodialysis initiation and transplant planning. Pathology showed multifocal clear cell RCC, ISUP grades 2–3. This case illustrates an atypical VHL presentation, where early syndromic suspicion and multidisciplinary coordination enabled timely and effective management.