Department of Orthopedics, University of Bologna, Istituto Ortopedico Rizzoli, Bologna, Italia.
Skeletal Radiol. 2011 Nov;40(11):1391-7. doi: 10.1007/s00256-010-1051-9. Epub 2010 Oct 25.
Gorham-Stout disease (also known as "disappearing bone disease") was first described by Jackson in 1838, but was properly defined by Gorham and Stout in a series of 24 patients in 1954-1955. It is a rare disease of unknown etiology (about 200 cases reported in the literature) characterized by spontaneous progressive resorption of bone without malignant proliferation of vascular structures. The diagnosis is one of exclusion and it is based on combined histological, radiological, and clinical features. Benign vascular proliferation with fatty bone marrow and thinning of bony trabeculae is a typical histological feature. Standard radiographs of disappearing bone disease show progressive bony resorption with adjacent soft tissue involvement. Most cases of Gorham-Stout disease resolve spontaneously, but prognosis remains unpredictable. This study reports 13 cases of Gorham-Stout disease treated in our institution from 1968 to 2008. The aim of the work was to review our series and the literature on this rare disease, as well as to evaluate whether or not an optimal treatment can be identified and recommended.
戈勒姆-斯托特病(也称为“消蚀性骨病”)于 1838 年由杰克逊首次描述,但在 1954-1955 年戈勒姆和斯托特对 24 例患者的一系列研究中得到了正确定义。它是一种病因不明的罕见疾病(文献中报道约 200 例),其特征为骨自发性进行性吸收,而血管结构无恶性增殖。诊断为排除性诊断,基于组织学、影像学和临床特征综合判断。良性血管增殖伴脂肪骨髓和骨小梁变薄是其典型的组织学特征。消蚀性骨病的标准 X 线片显示进行性骨吸收伴相邻软组织受累。大多数戈勒姆-斯托特病病例可自发缓解,但预后仍不可预测。本研究报告了我院自 1968 年至 2008 年收治的 13 例戈勒姆-斯托特病患者。目的是回顾该罕见疾病的我们的病例系列和文献,并评估是否可以确定和推荐最佳治疗方案。