Von Hippel-Lindau syndrome is associated with which vascular lesion?

• A. Capillary hemangioma
• B. Lymphangioma
• C. Progenic Granuloma
• D. Cavernous hemangioma

Answer: (D)

Von Hippel-Lindau disease is best understood through its association with highly vascular tumors, especially hemangioblastomas in the central nervous system and retina. These lesions are characterized by a proliferation of capillary vessels with a distinctive stromal component, so the vascular change most fitting to VHL is capillary-type hemangioblastoma. Cavernous hemangiomas, built from large, dilated vascular spaces, are a different type of vascular malformation and are not the classic vascular tumor you classically see with VHL. Lymphangiomas are lymphatic malformations, and pyogenic granulomas are reactive capillary proliferations, neither of which capture the typical VHL-associated vascular neoplasm.