van der Linden-van der Zwaag Henrica, Onvlee Gerard J
Orthopaedic Department, Leiden University Medical Center, Leiden, The Netherlands.
Acta Orthop Belg. 2006 Jun;72(3):261-8.
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.
戈勒姆巨大骨溶解症是特发性骨溶解的五种经典类型之一。股骨部位受累较为罕见。诊断基于病史资料（非遗传性）、生化资料（无肾病）、影像学资料（进行性单中心骨溶解且无骨膜反应）以及组织学资料（骨内血管瘤，伴有毛细血管或淋巴管，或两者皆有；最终出现纤维化）。如今，治疗主要包括截肢或关节成形术，并结合放疗。疾病偶尔会自发停止，但这无法预测。基因治疗在调节破骨细胞活性方面的潜在作用有待未来确定。文献回顾共发现22例股骨部位发生巨大骨溶解症的戈勒姆巨大骨溶解症病例，其中包括1例本人的病例。
