Haemangiopericytoma

This is largely a diagnosis of exclusion. Many tumours may show areas where the vasculature has a "pericytomatous" pattern, including solitary fibrous tumour, synovial sarcoma, infantile myofibromatosis, low-grade endometrial stromal tumour, mesenchymal chondrosarcoma, deep benign fibrous histiocytoma, myopericytoma and infantile fibrosarcoma. See the differential diagnosis of of spindle cell tumours of the pleura.

Immunohistochemistry

Insulin-like growth factor may be expressed: its diagnostic utility is being investigated.

Differential diagnosis

In particular, cases of metastatic haemangiopericytoma probably represetn one of the following:

• meningeal haemangiopericytomas are the best established group

• solitary fibrous tumour: so-called lipomatous haemangiopericytomas are CD34-positive and are probably a variant of solitary fibrous tumour.

• synovial sarcoma

• most infantile haemangiopericytoma are now considered to be myofibromatosis1

• orbital haemangiopericytoma overlaps with benign fibrous histiocytoma1

• sinonasal haemangiopericytoma is probably a variant of glomus tumour1

• myopericytoma

• mesenchymal chondrosarcoma

• low-grade endometrial stromal sarcoma.

Prognosis

Soft tissue haemangiopericytomas: recurrence rate 22-50%, , metastases in up to 60%1

Meningeal haemangiopericytomas: recurrence in 60%, metastases in 23%1

References

Diagnostic histopathology of tumors. Edited by CDM Fletcher. 2nd edition. Churchill Livingstone. Page 77.

Diagnostic Immunohistochemistry edited by Professor D. J. Dabbs, pages 76

1Tse, L.L. and Chan, J.K. Sinonasal haemangiopericytoma-like tumour: a sinonasal glomus tumour or a haemangiopericytoma? Histopathology 2002;40:510-7.

This page last revised 3.4.2003.

©SMUHT/PW Bishop