Cerebellar Hemangioblastoma

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51 year old male with a left cerebellar mass. What is the diagnosis?

Figure1. Left cerebellar Mass. Diagnosis?

Figure1.A: MRI- T2 weighted axial image shows cystic lesion in left cerebellar hemisphere with mass effect (green arrow). There is hydrocephalus (not shown). B: MRI- T1 weighted axial image shows cystic lesion in left cerebellar hemisphere with mass effect. There is an enhancing mural nodule with small serpentine flow voids (green arrow).

C: MRI- T1 sagittal image shows cystic lesion in left cerebellar hemisphere with mass effect. There is an enhancing mural nodule (green arrow).

Approximately 20-25% of people with cerebellar hemangioblastomas have von Hippel–Lindau disease, and as in this case, the majority (75-80%) are sporadic. 44-72% of patients with von Hippel–Lindau (vHL disease have at least one cerebellar hemangioblastoma, and 13-59% have at least one spinal hemangioblastoma. 80% of people with spinal hemangioblastomas have vHL disease. When associated with vHL disease, they occur at a younger age and have a worse prognosis.

The vast majority (95%) of intracranial hemangioblastomas occur in posterior fossa with 85% in the cerebellar hemisphere, 10% in the cerebellar vermis, and 5% in the medulla.

On imaging studies, hemangioblastomas may be solid, cystic, hemorrhagic, or mixed. Seen in about 60% of cases, the classic appearance is a cystic lesion with a solid enhancing mural nodule. About 40% of cases consist only of the enhancing nodule. Often there are serpentine flow voids within the nodule due to the highly vascular nature of the tumor. Surgical resection is usually curative, however, up to 25% of cases have subsequent local recurrence.

References:

1. Ho VB, Smirniotopoulos JG, Murphy FM et-al. Radiologic-pathologic correlation: hemangioblastoma. AJNR Am J Neuroradiol. 13 (5): 1343-52.

2. Leung RS, Biswas SV, Duncan M et-al. Imaging features of von Hippel-Lindau disease. Radiographics. 28 (1): 65-79.

Kevin M. Rice, MD is the president of Global Radiology CME. He has served in many leadership roles including Chair of the Radiology Department and Chief of Staff at Valley Presbyterian Hospital in Los Angeles, California. He has been a radiologist with Renaissance Imaging Medical Associates since 2000. Dr. Rice has made several media appearances as part of his ongoing commitment to public education. Dr. Rice's passion for state of the art radiology and teaching includes acting as a guest lecturer at UCLA. In 2015, Dr. Rice and Natalie Rice founded Global Radiology CME to provide innovative radiology education at exciting international destinations, with the world's foremost authorities in their field. In 2016, Dr. Rice was nominated and became a semifinalist for a "Minnie" Award for the Most Effective Radiology Educator.

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