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Published on: April 12, 2024
Massive osteolysis (Gorham's disease) affecting the femur
Henrica van der Linden-van der Zwaag1, Gerard J Onvlee
1Orthopaedic Department, Leiden University Medical Center, Leiden, The Netherlands. Enrike@vander-linden.nl
Insights
Gorham's massive osteolysis is a rare condition affecting bone. This study reviews 22 cases of femoral Gorham's massive osteolysis, highlighting diagnostic criteria and current treatment challenges.
Area of Science:
- Orthopedics
- Radiology
- Pathology
Background:
- Gorham's massive osteolysis (GMO) is a rare idiopathic osteolysis.
- Femoral localization of GMO is exceptionally rare.
- Diagnosis relies on specific clinical, biochemical, radiological, and histological findings.
Purpose of the Study:
- To review the literature on femoral Gorham's massive osteolysis.
- To summarize diagnostic criteria and treatment modalities for GMO.
- To present a case of femoral GMO.
Main Methods:
- Literature review of Gorham's massive osteolysis cases.
- Analysis of diagnostic data: anamnesis, biochemistry, radiography, histology.
- Review of current treatment options including surgery and radiotherapy.
Main Results:
- Identified 22 cases of femoral Gorham's massive osteolysis in the literature, including one personal case.
- Confirmed diagnosis requires exclusion of hereditary and nephropathic causes.
- Histology typically shows intraosseous angiomatosis.
Conclusions:
- Femoral Gorham's massive osteolysis is a rare entity requiring a multi-faceted diagnostic approach.
- Current treatments like amputation, arthroplasty, and radiotherapy have limitations.
- Spontaneous remission is unpredictable; future gene therapy roles require investigation.
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.
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