Epithelioid hemangioendothelioma – A rare pulmonary tumor

Abstract

ResumoOs autores descrevem um caso de uma neoplasia pulmonar rara -hemangioendotelioma epitelióide -numa doente do sexo feminino, de 39 anos, assintomática até Dezembro de 2003, altura em que apresentou toracalgia direita de características pleuríticas. A doente era portadora de uma radiografia torácica antiga, efectuada há 13 anos, que revelava múltiplos pequenos nódulos pulmonares bilaterais, atribuídos a sequelas de tuberculose pulmonar. O diagnóstico histológico definitivo foi efectuado através de biópsia pulmonar por toracotomia. Dado AbstractThe authors report a case of a rare pulmonary neoplasm -epithelioid hemangioendothelioma, in a 39 year-old woman, asymptomatic until December 2003, when she developed pleuritic and right-sided chest pain. The patient presented a previous chest radiograph, performed 13 years before, which showed multiple small bilateral pulmonary nodules attributed to tuberculous sequelae. The definitive histological diagnosis was made by lung biopsy through thoracotomy. The patient developed a clinical and Hemangioendotelioma epitelióide -Um tumor pulmonar raro Epithelioid hemangioendothelioma -A rare pulmonary tumorRecebido para publicação/received for publication: 07.12.14 Aceite para publicação/accepted for publication: 08.01.30

Multiple organ metastases of pulmonary epithelioid haemangioendothelioma and a review of the literature

“…The rarity of this condition, the lack of clear standards for treatment, and the partial-to-complete spontaneous regression of EHE seen in some patients up to 15 years from initial detection makes it difficult to decide on the most appropriate treatment. This is a rare disease, with approximately 100 cases described in the English literature [7][8][9][10][11][12][13][14][15][16][17][18][19][20][21] (Table 1) and approximately 10 cases reported in China [22,23].…”

Epithelioid hemangioendotheliomas of the liver and lung in children and adolescents

“…The use of the thalidomide derivative lenalidomide resulted in SD in three of three cases reported . The pleiotropic cytokine interferon‐alpha yielded a CR in one, PR in one, and SD in two of six cases reported; two patients progressed on interferon‐alpha . Finally, the multityrosine kinase inhibitor pazopanib achieved one PR and one SD in two cases reported .…”

“…[46][47][48] The pleiotropic cytokine interferon-alpha yielded a CR in one, PR in one, and SD in two of six cases reported; two patients progressed on interferon-alpha. [49][50][51][52][53][54] Finally, the multityrosine kinase inhibitor pazopanib achieved one PR and one SD in two cases reported. 55,56 Therapy was overall well tolerated.…”