Genitourinary Radiology > Kidneys > Parenchymal > Large Kidney > Von Hippel-Lindau


Von Hippel-Lindau

 

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 extraadrenal.

Findings highly suspicious of vHL include multiple simple renal cysts in combination with renal cell carcinoma and multiple pancreatic cysts.

MRI of the kidneys (A=T2 wieghted coronal, B = T1 wieghted coronal, A=T1 wieghted coronal with gadolinium) shows a cystic mass in lower pole of right kidney (arrow). There are mural nodules within the cyst. With gadolinium, to the nodular is seen to enhance consistent with a bosniak IV renal cell carcinoma. (Click here for Bosniak classification of renal cysts). Did you note the parenchyma in the lower pole of the left kidney is lobular and demonstrates abnormal enhancement (*). This was a synchronous solid renal cell carcinoma in this 37 year old male with vHL.


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