title: Massive osteolysis (Gorham's disease) affecting the femur.
abstract: Gorham's massive osteolysis is one of the five classical types of idiopathic osteolysis. The femoral localisation is rare. The diagnosis is based on anamnestic data (non-hereditary), on biochemical data (absence of nephropathy), on radiographical data (progressive monocentric osteolysis without periosteal reaction), and on histological data (intraosseous angiomatosis with either capillaries or lymph vessels, or both; eventually fibrosis). Nowadays, treatment mostly consists of amputation or arthroplasty, combined with radiotherapy. Spontaneous arrest of the disease occasionally occurs, but this is unpredictable. The possible role of gene-therapy in the regulation of osteoclastic activity has to be determined in the future. Review of the literature produced 22 cases of Gorham's massive osteolysis with a localisation at the femur massive osteolysis, including one personal case.
authors.affiliation: Orthopaedic Department, Leiden University Medical Center, Leiden, The Netherlands. Enrike@vander-linden.nl
authors.forename: Henrica
authors.initials: H
authors.lastname: van der Linden-van der Zwaag
authors.forename: Gerard J
authors.initials: GJ
authors.lastname: Onvlee
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D002648:Child; D002675:Child, Preschool; D005260:Female; D005269:Femur; D006801:Humans; D007223:Infant; D008297:Male; D008875:Middle Aged; D010015:Osteolysis, Essential / Q000175:diagnosis / Q000628:therapy