title: Sirolimus on Gorham-Stout disease. Case report.
abstract: BACKGROUND
  Gorham-Stout disease (GSD) is a rare disease of unknown etiology characterized by vascular proliferation that produces destruction of bone matrix.
  
  
  METHODS
  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 6thand 10thleft ribs archs. Later, a thoracotomy was performed observed absence of the end of the 6thand 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.
  
  
  RESULTS
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Departamento de Medicina Interna. Hospital Clínico Universitario de Salamanca. Salamanca. España.
authors.forename: Vanessa
authors.initials: V
authors.lastname: García
authors.affiliation: Departamento de Medicina Interna. Hospital Clínico Universitario de Salamanca. Salamanca. España.
authors.forename: Gloria
authors.initials: G
authors.lastname: Alonso-Claudio
authors.affiliation: Departamento de Cirugía Torácica. Hospital Clínico Universitario de Salamanca. Salamanca. España.
authors.forename: M-Teresa
authors.initials: MT
authors.lastname: Gómez-Hernández
authors.affiliation: Departamento de Medicina Interna. Hospital Clínico Universitario de Salamanca. Salamanca. España.
authors.forename: Antonio-J
authors.initials: AJ
authors.lastname: Chamorro
mesh_terms: D000328:Adult; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D010015:Osteolysis, Essential / Q000175:diagnosis / Q000188:drug therapy* / Q000503:physiopathology; D010996:Pleural Effusion / Q000188:drug therapy / Q000209:etiology; D020123:Sirolimus / Q000627:therapeutic use*; D016896:Treatment Outcome
chemical_list: D007166:Immunosuppressive Agents; D020123:Sirolimus
keywords: Pleural effusion; osteolysis essential, metrorrhagia, sirolimus, Gorham-Stout disease, prognosis, rare diseases, cell proliferation.