A Rare Case of Orbital Haemangiopericytoma Arising in Childhood

Abstract

Haemangiopericytoma (HPC) is a rare soft tissue tumour of fibroblastic origin and is part of the solitary fibrous tumour spectrum. The tumour is generally considered to be benign, but can behave clinically as if sarcomatous -- with relentless infiltrative local growth. HPC generally presents in adulthood (median age 45 years for orbital disease) and is equally frequent in both sexes. HPC can arise in any site in the body and presents as a slowly growing, painless mass. We report a case of a 20 year old African male seen at Kikuyu Eye Unit, Kenya, with a 12 year history of a gradually enlarging, painless orbital mass. The patient underwent skin-sparing orbital exenteration with complete tumour excision; histology confirmed diagnosis of HPC.

• Haemangiopericytoma
• orbital tumour
• paediatric orbital tumour
• solitary fibrous tumour