A 32-year-old man with von Hippel-Lindau disease undergoes MRI for progressive headache and ataxia. The posterior fossa study shows a large cystic cerebellar hemispheric lesion containing an intensely, uniformly enhancing mural nodule adjacent to which prominent flow voids are seen. The most likely diagnosis is:

• A Pilocytic astrocytoma
• B Dermoid cyst with rupture
• C Metastatic renal cell carcinoma
• D Hemangioblastoma ✓

Explanation

Hemangioblastoma is the hallmark CNS tumor of von Hippel-Lindau disease, occurring in adults as a cystic cerebellar lesion with a richly vascular, intensely enhancing mural nodule; flow voids reflect its hypervascular nature. Pilocytic astrocytoma can look similar but occurs in children, lacks flow voids, and is not linked to VHL. Metastases are multiple and enhance diffusely rather than as a single mural nodule.

Reference: Robbins and Cotran Pathologic Basis of Disease, 10th ed.

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

Written and medically reviewed by the StethoPrep medical team.