Sirolimus on Gorham-Stout disease. Case report.

Colombia Medica (Online)

María Teresa Gómez-Hernández¹  Vanessa Garcia²  Gloria Alonso-Claudio²  Antonio-J Chamorro² 

[1]Hospital Clínico Universitario de Salamanca. Salamanca. España

[2]Hospital Clínico Universitario de Salamanca. Salamanca. España.

关键词: Pleural effusion;    osteolysis essential;    metrorrhagia;    sirolimus;    Gorham-Stout disease;    prognosis;    rare diseases;    cell proliferation.;   

DOI  :  

学科分类：医学（综合）

来源: Universidad del Valle * Facultad de Salud / Universidad del Valle, Faculty of Health

【 摘 要 】

Background:Gorham-Stout disease (GSD) is a rare disease of unknown etiologycharacterized by vascular proliferation that produces destruction ofbone matrix.Case description:This case is about 43 year old woman who begins with pain in sternum,dyspnea, abdominal mass and, serous-hematic pleural effusion. Imagingtests were performed showing lesions on 6 thand 10 thleft ribs archs. Later, a thoracotomy was performed observed absence of the end of the 6 thand lung, pleural and costal biopsy was token. The histologic featuresdescribed lymphatic vascular proliferation in bone tissue of chest wall.Other pathologies were excluded and in view of the findings, GSDdiagnosis was made.Treatment and outcome:treatment was initiated with sirolimus achieving remission of thedisease after the first month; however, because the presence ofmetrorrhagia the treatment was discontinued, reappearing symptomsafterwards. For that reason the treatment was restarted gettingdisappearance of the symptoms again, 4 weeks later.Clinical relevance:we present the first clinical cases of EGS with pleural effusion withresponse to sirolimus treatment that could be an alternative to thecurrent therapy.

【 授权许可】

CC BY