Massive osteolysis (Gorham's disease) affecting the femur.
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.
van der Linden-van der Zwaag
Henrica
H
Orthopaedic Department, Leiden University Medical Center, Leiden, The Netherlands. Enrike@vander-linden.nl
Onvlee
Gerard J
GJ
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