title: 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.
authors.affiliation: Department of Genetics, UCL Institute of Ophthalmology, London , EC1V 9EL , United Kingdom .
authors.forename: Anna M
authors.initials: AM
authors.lastname: Rose
authors.forename: Joy
authors.initials: J
authors.lastname: Kabiru
authors.forename: Geoffrey E
authors.initials: GE
authors.lastname: Rose
mesh_terms: D000199:Actins / Q000378:metabolism; D018952:Antigens, CD34 / Q000378:metabolism; D014408:Biomarkers, Tumor / Q000378:metabolism; D003185:Complement C8 / Q000378:metabolism; D006393:Hemangiopericytoma / Q000378:metabolism / Q000473:pathology* / Q000601:surgery; D006801:Humans; D008297:Male; D017233:Orbit Evisceration; D009918:Orbital Neoplasms / Q000378:metabolism / Q000473:pathology* / Q000601:surgery; D055815:Young Adult
chemical_list: C541116:ACTA2 protein, human; D000199:Actins; D018952:Antigens, CD34; D014408:Biomarkers, Tumor; D003185:Complement C8