Massive osteolysis of the femur (Gorham's disease) : a case report and review of the literature
Mendez, A.A.; Keret, D.; Robertson, W.; MacEwen, G.D.
Journal of Pediatric Orthopedics 9(5): 604-608
1989
ISSN/ISBN: 0271-6798 PMID: 2677046 Document Number: 326720
A case of Gorham's disease involving the proximal femur of a 12-year-old girl is presented. Although Gorham's disease is rare and has benign histologic characteristics, in this patient it took an aggressive clinical course that resulted in severe morbidity. Diagnosis is difficult to establish; almost all investigative approaches provide only indirect evidence of the pathology. A high index of suspicion and open biopsy performed soon after presentation will establish diagnosis. Treatment modalities may be more effective if applied earlier in the disease process, especially as the osteolytic process appears to escalate after pathologic fractures, which occur frequently in this disorder.