A 38-year-old man with von Hippel-Lindau disease undergoes MRI brain for headache. A large cystic mass is seen in the right cerebellar hemisphere with a strongly enhancing nodule along its wall and no significant surrounding oedema. What is the most likely diagnosis?
- A Pilocytic astrocytoma
- B Medulloblastoma
- C Cerebellar hemangioblastoma ✓
- D Arachnoid cyst
Explanation
Hemangioblastoma is the hallmark cerebellar tumor of von Hippel-Lindau disease and shows the classic 'cyst with a mural nodule' pattern, where the intensely enhancing nodule contains the tumor and the cyst wall is non-neoplastic compressed brain. Pilocytic astrocytoma can look identical but occurs in children and is not associated with VHL. Medulloblastoma is a solid midline tumor in children with restricted diffusion, and an arachnoid cyst does not enhance.
Reference: Robbins and Cotran Pathologic Basis of Disease, 10th ed.
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Written and medically reviewed by the StethoPrep medical team.