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Vol. 15. Issue 6.
Pages 1167-1174 (November - December 2009)
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Vol. 15. Issue 6.
Pages 1167-1174 (November - December 2009)
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Hemangioendotelioma epitelióide do pulmão – Raridade, dificuldades diagnósticas e terapêutica
Pulmonary epithelioid hemangioendothelioma – Rarity, diagnosis and treatment difficulties
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Telma Lopes1,7,
Corresponding author
telmasl.81@gmail.com

Centro Hospitalar de Lisboa Norte (CHLN) – Hospital de Pulido Valente Alameda das Linhas de Torres, n.° 117 1769-001 Lisboa.
, Susana Clemente2,7, Amélia Feliciano3,7, Isabel Lourenço4,9, Agostinho Costa9,8, Joaquim Gil Duarte5,7, Pina Jaime6, Ramos Dra. Madalena9
1 Interna da Formação Específica de Pneumologia
2 Assistente Hospitalar de Pneumologia
3 Assistente Hospitalar Graduada de Anatomia Patológica
4 Assistente Hospitalar Graduado de Pneumologia
5 Chefe de Serviço de Pneumologia
6 Departamento de Pneumologia
7 Serviço de Pneumologia IV
8 Unidade de Oncologia Pneumológica
9 Serviço de Anatomia Patológica. Chefe de Serviço
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Resumo

Os autores apresentam um caso de hemangioendotelioma epitelióide (HEE) primário do pulmão num doente de 51 anos, sexo masculino, que iniciou queixas de tosse seca, seguida de sintomas constitucionais e dispneia. Após realização de alguns exames complementares de diagnóstico, que incluíram biópsia cirúrgica, foi considerada a hipótese de tuberculose pulmonar e iniciou antibacilares, que manteve durante três semanas. Por agravamento clínico e imagiológico, foi feita revisão do caso e estudo imunoistoquímico dos tecidos pulmonares biopsados e concluiu-se estar perante um HEE de alto grau/angiossarcoma do pulmão. Iniciou quimioterapia com carboplatina, etoposido e bevacizumab, com ligeira melhoria. Faleceu sete meses após o aparecimento dos primeiros sintomas e sete semanas após o diagnóstico definitivo.

Os autores pretendem realçar a raridade desta neoplasia pulmonar, a importância da suspeição clínica e as dificuldades no diagnóstico e tratamento, e salientar os potenciais benefícios da utilização de um fármaco antiangiogénico.

Palavras-chave:
Hemangioendotelioma epitelióide
angiossarcoma do pulmão
asbestos
bevacizumab
Abstract

The authors report a case of a primary pulmonary epithelioid haemangioendothelioma (EHE) in a 51 year-old man, a mechanic, who complained of a dry cough followed by constitutional symptoms and dyspnoea. Patient underwent a series of diagnostic exams including surgical biopsy and pulmonary tuberculosis was diagnosed. He was prescribed tuberculosis drugs for three weeks. Following clinical and imagiology deterioration, the case was reviewed by pathologists who concluded the pulmonary biopsy revealed an intermediate/high grade pulmonary EHE/angiosarcoma. The patient underwent three cycles of chemotherapy with carboplatin, etoposide and bevacizumab with no complications. He died seven months after onset of symptoms and seven weeks after definitive diagnosis.

The authors wish to highlight the rarity of this pulmonary neoplasm and the importance of clinical suspicion, and the diagnosis and treatment difficulties in addition to the potential benefits of antiangiogenic drugs.

Key-words:
Epithelioid hemangioendothelioma
pulmonary angiosarcoma
asbestos
bevacizumab
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Copyright © 2009. Sociedade Portuguesa de Pneumologia
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