Strahlenklinik, Klinikum Offenbach, Offenbach am Main, Germany.
Strahlenther Onkol. 2011 Feb;187(2):140-3. doi: 10.1007/s00066-010-2174-6. Epub 2011 Jan 18.
The Gorham-Stout syndrome (GSS) is a rare, benign idiopathic and progressive disorder causing massive osteolysis due to a vascular hyperproliferation replacing the bony structure. Clinical experience concerning the efficacy of radiation therapy (RT) is limited to about 50 of an overall 200 cases reported worldwide.
A 24-year-old bedridden woman had histologically proven GSS with destruction of the anterior pelvic girdle and received RT for a total dose of 45.0 Gy applied in 5 weekly fractions of 1.8 Gy. In addition, the patient received intravenously 4 mg zoledronic acid once a month. One year after the combined treatment, complete pain relief occurred, and the patient was able to walk without the use of appliances. Imaging studies revealed no progression of the osteolysis but only minimal signs of remineralization.
Combined treatment with RT and bisphosphonate administration can prevent the progression of osteolysis in GSS. Total doses of 40-45 Gy are recommended.
Gorham-Stout 综合征(GSS)是一种罕见的良性特发性进行性疾病,由于血管过度增生取代骨结构而导致大量骨质溶解。关于放射治疗(RT)疗效的临床经验仅限于全世界报告的 200 例中的约 50 例。
一名 24 岁卧床不起的女性患有组织学证实的 GSS,骨盆前带破坏,并接受了总剂量为 45.0 Gy 的 RT,每周 5 次,每次 1.8 Gy。此外,患者每月接受静脉注射唑来膦酸 4 毫克。联合治疗一年后,完全缓解疼痛,患者无需使用器械即可行走。影像学研究显示骨质溶解没有进展,只有轻微的再矿化迹象。
RT 和双膦酸盐联合治疗可预防 GSS 中骨质溶解的进展。建议使用总剂量为 40-45 Gy。