Schumann E, Wild A, Seller K
Orthopädische Klinik, Universitätsklinikum Leipzig.
Z Orthop Unfall. 2008 Sep-Oct;146(5):655-9. doi: 10.1055/s-2008-1038798. Epub 2008 Oct 9.
Gorham-Stout disease is a rare entity characterised by a massive osteolysis and typically starts monocentrically in an isolated bone. Concerning the pathogenesis, haemangiomatosis and an overstimulation of osteoclasts are discussed which cause a progressive destruction of the affected skeleton and involve neighbouring soft tissue in the further progress of the disease.
This report is about a 45-year-old male who suffered a traumatic insult. X-rays showed an osteolytic lesion in the 4th lumbar vertebra with depression in the ventral roof plate. Further radiological investigation has ruled out a malignant lesion and suggested Gorham-Stout disease as a diagnosis. Therapy has been carried out using bisphosphonates as well as fractioned radiation of the vertebra with 30 Gy under which a remission was achieved.
The progress of our case correlates with published data in the literature. Spinal lesions are rare, but possible neurological complications or a possible chylothorax reflect the high mortality rate. We found no complications in our case.
Despite massive osteolysis with resorption of up to a whole bone segment, radiation with a dose of 22.8 Gy and the use of antiresorptive substances, as reported in our case, have often been shown to limit the destructive progression of the disease. A causal therapy of the disease is not known yet.
戈勒姆-斯托特病是一种罕见疾病，其特征为大量骨质溶解，通常始于单一骨骼的单中心发病。关于其发病机制，有血管瘤病和破骨细胞过度刺激的讨论，这会导致受影响骨骼的渐进性破坏，并在疾病进一步发展过程中累及邻近软组织。
本报告讲述一名45岁男性遭受创伤性损伤。X线显示第四腰椎有骨质溶解病变，腹侧顶板凹陷。进一步的影像学检查排除了恶性病变，并诊断为戈勒姆-斯托特病。采用双膦酸盐以及对椎体进行30 Gy的分次放疗进行治疗，实现了病情缓解。
我们病例的进展与文献中已发表的数据相关。脊柱病变罕见，但可能出现的神经并发症或乳糜胸反映出高死亡率。我们的病例未发现并发症。
如我们病例中所报告的，尽管存在高达整个骨段吸收的大量骨质溶解，但22.8 Gy的放疗和抗吸收物质的使用常常显示出可限制疾病的破坏性进展。该疾病的病因治疗尚不清楚。
