VonHippel-Lindau syndrome (VHL) is an autosomal dominant disorder characterized by a variety of benign and malignant neoplasms. Common tumors include hemangioblastoma, clear cell renal cell carcinoma, pheochromocytoma, serous cystadenoma and neuroendocrine tumors of the pancreas, hepatic adenoma, endolymphatic sac tumors of the middle ear, and papillary cystadenoma of the epididymis and broad ligament.75% of these patients have simple renal cysts. Renal cell carcinoma develops in 40%, 75% of which are multifocal. Pheochromocytoma occurs in 15%, and is often multiple, bilateral, and extra adrenal.
Findings highly suspicious of VHL include multiple simple renal cysts in combination with renal cell carcinoma and multiple pancreatic cysts.
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