Sato K, Sugiura H, Yamamura S, Mieno T, Nagasaka T, Nakashima N
Department of Orthopaedics, Nagoya University School of Medicine, Japan.
Arch Orthop Trauma Surg. 1997;116(8):510-3. doi: 10.1007/BF00387590.
Gorham syndrome (massive osteolysis) is a very rare tumour-like lesion characterized by progressive osteolysis. The diagnosis must be confirmed by the microscopic finding of intramedullary angioma-like vascular structures. We report a case of a 15-year-old boy with a pathological fracture in his left humerus. Imaging modalities such as magnetic resonance imaging, computed tomography, angiography and bone scintigraphy failed to disclose to tumorous lesion that filled a cavity in the left humerus. After observing the boy's progress for 6 months, a temporary diagnosis of Gorham syndrome was made, and surgical treatment was chosen. After resection of the left humeral head and the proximal one-quarter of the humerus, thorough curettage was performed in the distal humerus and an intramedullary artificial humeral head fixed with adequate success. Pathological examination of the specimen revealed intramedullary haemangioma of the humerus.
戈勒姆综合征(大块骨质溶解症)是一种非常罕见的肿瘤样病变,其特征为进行性骨质溶解。诊断必须通过骨髓内血管瘤样血管结构的显微镜检查结果来证实。我们报告一例15岁男孩,其左肱骨发生病理性骨折。磁共振成像、计算机断层扫描、血管造影和骨闪烁显像等影像学检查均未能发现填充左肱骨腔内的肿瘤性病变。在观察该男孩6个月的病情进展后,做出了戈勒姆综合征的临时诊断,并选择了手术治疗。切除左肱骨头和肱骨近端四分之一后,对肱骨远端进行了彻底刮除,并成功固定了髓内人工肱骨头。标本的病理检查显示为肱骨骨髓内血管瘤。