García Vanessa, Alonso-Claudio Gloria, Gómez-Hernández M-Teresa, Chamorro Antonio-J
Departamento de Medicina Interna. Hospital Clínico Universitario de Salamanca. Salamanca. España.
Departamento de Cirugía Torácica. Hospital Clínico Universitario de Salamanca. Salamanca. España.
Colomb Med (Cali). 2016 Dec 30;47(4):213-216.
Gorham-Stout disease (GSD) is a rare disease of unknown etiology characterized by vascular proliferation that produces destruction of bone matrix.
This case is about 43 year old woman who begins with pain in sternum, dyspnea, abdominal mass and, serous-hematic pleural effusion. Imaging tests were performed showing lesions on 6 and 10 left ribs archs. Later, a thoracotomy was performed observed absence of the end of the 6 and lung, pleural and costal biopsy was token. The histologic features described lymphatic vascular proliferation in bone tissue of chest wall. Other pathologies were excluded and in view of the findings, GSD diagnosis was made.
treatment was initiated with sirolimus achieving remission of the disease after the first month; however, because the presence of metrorrhagia the treatment was discontinued, reappearing symptoms afterwards. For that reason the treatment was restarted getting disappearance of the symptoms again, 4 weeks later.
we present the first clinical cases of EGS with pleural effusion with response to sirolimus treatment that could be an alternative to the current therapy.
戈勒姆-斯托特病（GSD）是一种病因不明的罕见疾病，其特征为血管增生导致骨基质破坏。
该病例为一名43岁女性，最初出现胸骨疼痛、呼吸困难、腹部肿块及浆液血性胸腔积液。影像学检查显示左侧第6和第10肋弓有病变。随后进行了开胸手术，发现第6肋末端缺失及肺部、胸膜和肋骨活检。组织学特征显示胸壁骨组织中有淋巴管增生。排除了其他病理情况，根据检查结果，诊断为GSD。
开始使用西罗莫司治疗，第一个月后病情缓解；然而，由于出现子宫出血，治疗中断，之后症状再次出现。因此重新开始治疗，4周后症状再次消失。
我们报告了首例伴有胸腔积液且对西罗莫司治疗有反应的EGS临床病例，这可能是当前治疗的一种替代方法。
