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Hemangioblastoma

Case contributed by Assoc Prof Frank Gaillard

Presentation

History of chronic lymphocytic leukemia and 3 x melanoma resections.

Patient Data

Age: 65 years
Gender: Male
CT

A rounded, vividly and homogenous enhancing mass in the superior left cerebellum with surrounding parenchymal hypoattenuation compatible with edema, unchanged from the previous study. Local mass effect with effacement of the cerebellar folia, and partial effacement and displacement to the right of the fourth ventricle. Slight descent of the cerebellar tonsils through the foramen magnum (3 mm). The third and lateral ventricles remain of normal size with no evidence of obstructive hydrocephalus at this point in time. No other lesion is identified. No intra or extra-axial hemorrhage. The dural venous sinuses are patent.

MRI

A vividly enhancing mass in the left cerebellar hemisphere has associate prominent vessels (best seen as flow-voids on T2 and coronal FLAIR) and local mass-effect without hydrocephalus. It is difficult to categorically place this lesion as either intra- or extra-axial. It abuts the dura with only a small area of contact and no dural tail. No other lesions identified. 

Although in this clinical setting a melanoma metastasis must be included in the differential, absence of hemorrhage or intrinsic high T1 signal and presence of large vessels strongly favor an alternative diagnosis, namely a hemangioblastoma. 

Case Discussion

The patient went on to have a resection. 

Histology

Sections show tumor composed of sheets of cells with eosinophilic cytoplasm with ovoid to spindled nuclei with evenly distributed chromatin with many interspersed small and larger thin-walled blood vessels. These cells display mild nuclear pleomorphism. Nucleoli and mitoses are not seen.

The tumor cells are positive for inhibin and negative for EMA, CD10 and PAX8.

FINAL DIAGNOSIS: hemangioblastoma (WHO grade I).

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Case information

rID: 68994
Published: 15th Apr 2020
Last edited: 22nd Oct 2020
Inclusion in quiz mode: Included

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