Hemangioblastoma

Case contributed by A.Prof Frank Gaillard

Presentation

Headache.

Patient Data

Age: 50 years
CT

A right-sided posterior fossa mass with a vividly enhancing component is associated with extensive edema and mass effect. 

MRI

An irregular mixed solid and cystic lesion in the right cerebellum which abuts the posterolateral pial surface with surrounding T2 and FLAIR hyperintensity consistent with edema. There is associated tonsillar herniation. Ventricles are slightly enlarged although the fourth ventricle is compressed and distorted but not completely effaced. The adjacent transverse and sigmoid dural venous sinus appears intact although dedicated sequences have not been performed. A few mildly prominent vessels are noted adjacent to the lesion. Marginal susceptibility is due at least in part to marginal vessels, however a minor component of hemorrhage is also suspected. The solid component shows vivid enhancement on CT and MRI and also demonstrates markedly increased CBV without diffusion restriction. Minor reversal of choline:creatinine peaks on MR spectroscopy with elevated lipid /lactate.

Conclusion: Findings are suggestive of right cerebellar hemangioblastoma.

Case Discussion

The patient went on to have a resection.

Histology

Sections of cerebellum show a moderate cellular tumor composed of nests of tumor cells separated by variably sized thin-walled blood vessels. Tumor cells demonstrate biphasic morphology with abundant eosinophilic to clear cytoplasms, oval nuclei with coarse granular chromatin and small nuceloli. No necrosis or mitoses are seen.

Immunohistochemically tumor cell stain:

  • Inhibin: positive
  • S100: positive
  • CAM5.2: negative
  • PAX8: negative
  • CD10: negative

FINAL DIAGNOSIS: hemangioblastoma (cellular variant) - WHO Grade I.

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Case information

rID: 55638
Published: 2nd Jun 2018
Last edited: 10th Jan 2019
Inclusion in quiz mode: Included

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