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Cerebellar Hemangioblastoma

October 29, 2015

Headache and Dizziness • Xray of the Week 2016 • Week #28

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

Left: MRI- T2 weighted axial image shows cystic lesion in left cerebellar hemisphere with mass effect. There is hydrocephalus (not shown).
Middle: 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.

Right: MRI- T1 sagittal image shows cystic lesion in left cerebellar hemisphere with mass effect. There is an enhancing mural nodule.

 

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 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 serves as the Medical Director of the Radiology Department of Valley Presbyterian Hospital in Los Angeles, California and is a radiologist with Renaissance Imaging Medical Associates. 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 launched Global Radiology CME to provide innovative radiology education at exciting international destinations, with the world's foremost authorities in their field.

 

Follow Dr. Rice on Twitter @KevinRiceMD

 

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