Haemangiopericytoma

Case contributed by A.Prof Frank Gaillard

Presentation

Headache

Patient Data

Age: 25 years
CT

A large lobulated mass is seen centered on the falx, with prominent positive mass effect and surrounding vasogenic oedema. The mass is heterogeneous in density with much of it being somewhat hyperdense. 

MRI

A large mass arising from the falx is noted, with 'destruction' of the falx. The mass is multilobulated, and has numerous peripheral flow voids. Areas of haemorrhage and cystic change are noted. ADC demonstrates low values, consistent with hypercellular nature of the lesion. 

Conclusion

The differential is between a meningioma and a hemangiopericytoma. Presence large peripheral flow voids and multi lobulation and 'destruction' of the falx, favours the latter. 

Case Discussion

The patient went on to have surgery.

Histology

MICROSCOPIC DESCRIPTION: Sections show a hypercellular spindle cell tumour with prominent haemangiopericytomatous "staghorn" blood vessels. Tumour cells are arranged in solid sheets containing abundant pale eosinophilic cytoplasm, elongate oval moderately pleomophic nuclei with coarse chromatin and inconspicuous nucleoli. Focally the tumour demonstrates myxoid degeneration (specimen 5). No sarcomatous elements, necrosis or atypia is seen. There are up to 9 mitoses per 10 hpf identified.

Immunohistochemical results show tumour cells stain: CD34+ (focal), BCL2+, CD99+, STAT6+ (focal), EMA- and PgR-. The Topoisomerase proliferation index is approximately 5-10%.

FINAL DIAGNOSIS:

Haemangiopericytoma with anaplastic features (WHO grade III).

NB: as of the 2016 update to the WHO classification of CNS tumours, haemangiopericytomas are considered part of a spectrum along with solitary fibrous tumour of the dura. 

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Case information

rID: 47441
Case created: 16th Aug 2016
Last edited: 1st Sep 2016
Inclusion in quiz mode: Included

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