Von Hippel Lindau disease


Von Hippel-Lindau (vHL) disease is a multi-system disorder characterized by the development of numerous benign and malignant tumors as well as several non-malignant lesions.

The characteristic neurological tumor is a hemangioblastoma, which may involve the brain or spinal cord.

Renal cell carcinoma can occur in up to 40% of cases and is one of the most feared complications. RCC's present at an earlier age in those with vHL, as in this case in where the patient was in their 20's.

The renal lesion was confirmed as a cystic renal cell carcinoma on CT guided biopsy.