Von Hippel-Lindau disease
Discussion:
This patient had a right nephrectomy for renal cell carcinoma and left adrenalectomy for a pheochromocytoma.
The association of renal cell carcinoma, renal cysts, adrenal pheochromocytoma, cerebral, and spinal hemangioblastoma are most consistent with von Hippel Lindau disease (vHL).
The association of an extra-axial cerebral mass (most likely a solitary fibrous tumor (hemangiopericytoma) in this case) and von Hippel Lindau disease is rarely reported in the literature.
Additional contributor: R Bouguelaa, MD.
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- Hemangioblastoma (central nervous system)
- Hemangiopericytoma (historical)
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- Meningioma
- Obstructive hydrocephalus
- Pheochromocytoma
- Renal cell carcinoma
- Renal epithelial cyst
- Sclera
- Spinal hemangioblastoma
- Subarachnoid space
- Subfalcine herniation
- Von Hippel-Lindau disease
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