Unilateral Pheochromocytoma in Von Hippel-Lindau Syndrome Revealed by a Hemangioblastoma

Abstract

Von Hippel-Lindau (VHL) syndrome is characterized by the occurrence of both benign and malignant tumors, with typical manifestations such as cerebellar hemangioblastoma, Renal cell carcinoma (RCC) and pheochromocytomas.1 Additional tumors that may be associated include pancreatic cysts; neuroendocrine tumours; epididymal cysts and endolymphatic sac tumors.1 This case report details a 29-year-old male who arrived at the Emergency Department (ED) with a one-month history of headaches accompanied by dizziness and vomiting. A Computed Tomography (CT) scan of the head followed by Magnetic Resonance Imaging (MRI) suggested cerebellar hemangioblastoma. Further workup showed multiple tumors, including unilateral pheochromocytoma and pancreatic cysts, which aligned with a diagnosis of von Hippel-Lindau (VHL) syndrome. Notably, the patient's catecholamine and vanillylmandelic acid (VMA) levels were within normal limits. The patient underwent surgical intervention for the cerebellar hemangioblastoma, and the postoperative recovery was uneventful. This case is noteworthy due to its characteristic findings and the rarity of the condition.