Haemangioblastoma

Case contributed by A.Prof Frank Gaillard

Patient Data

Age: young adult
MRI

MRI of the posterior fossa of a young adult demonstrates a cystic mass in the superior aspect of the left cerebellar hemisphere. An excentric solid component is seen anterolaterally which is somewhat hypointense on T1 and hyperintense on T2 compared to adjacent brain. A minor degree of high T2 signal is seen surrounding the mass consistent with oedema.

Following administration of contrast the solid component enhances vividly , with no evidence of enhancement of the cyst wall. Features are consistent with a haemangioblastoma of cerebellum. (confirmed histologically)

No other lesions are identified to suggest underlying von Hippel Lindau syndrome. 

Case Discussion

See also

Histology

On sectioning of the larger specimen, it has an outer rim of dark red, friable tissue resembling clot with central soft red tissue resembling blood.

Microscopic Description

The sections show that the lesion is comprised of numerous thin-walled vessels. Between these vessels there are stromal cells, which have rounded nuclei. Some of the nuclei are hyperchromatic and large. No mitotic figures are seen. There is cerebellum, which shows atrophy as evidenced by rarefaction of the granular layer and loss of Purkinje cells. Granular PAS-positivity, removed by diastase digestion, is evident in stromal cells. The tumor shows numerous reticulin fibrils. Immunohistochemistry shows that the tumour is negative for glial fibrillary acidic protein, epithelial membrane antigen and keratin. There are numerous small-diameter CD31-positive vessels within the tumour.

PlayAdd to Share

Case information

rID: 4050
Case created: 15th Jul 2008
Last edited: 29th Mar 2017
Inclusion in quiz mode: Included

Updating… Please wait.
Loadinganimation

Alert accept

Error Unable to process the form. Check for errors and try again.

Alert accept Thank you for updating your details.