Publication: Unilateral Pheochromocytoma in Von Hippel-Lindau Syndrome Revealed by a Hemangioblastoma
Date
2024
Authors
Journal Title
Journal ISSN
Volume Title
Publisher
Kathmandu University
Abstract
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.
KEY WORDS
Hemangioblastoma, Pheochromocytoma, Von Hippel-Lindau syndrome
Description
KC S,1 Poudyal H,2 Baral S3
1Department of Radiology
Patan Academy of Health Sciences
Lalitpur, Nepal
2Department of Internal Medicine
Nepal APF Hospital
Balambu, Kathmandu, Nepal
3Resident, Department of Radiology
Patan Academy of Health Science
Lalitpur, Nepal
Keywords
Hemangioblastoma, Pheochromocytoma, Von Hippel-Lindau syndrome