Von Hippel-Lindau disease features include which extrarenal manifestation?

• A. Chronic pancreatitis
• B. Polycystic liver disease
• C. Amyloidosis
• D. Retinal hemangioblastoma

Answer: (D)

Retinal hemangioblastoma is a classic extrarenal manifestation of Von Hippel-Lindau disease. This hereditary condition predisposes to highly vascular tumors in multiple organs, with the retina and CNS being prominent sites outside the kidneys. Retinal hemangioblastomas can cause vision changes and are often a key clue that leads clinicians to diagnose the syndrome. That’s why this option best fits as an extrarenal manifestation of VHL.

The other options aren’t typical features of VHL. Pancreatitis isn’t a hallmark of the disease, even though VHL can involve the pancreas with cysts or neuroendocrine tumors. Polycystic liver disease is not a standard VHL feature, and amyloidosis is unrelated to VHL pathophysiology.