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Journal Information
Vol. 14. Issue 3.
Pages 421-425 (May - June 2008)
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Vol. 14. Issue 3.
Pages 421-425 (May - June 2008)
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Epithelioid hemangioendothelioma – A rare pulmonary tumor
Hemangioendotelioma epitelióide – Um tumor pulmonar raro
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Sandra Saleiro1,
Corresponding author
sandrasaleiro@portugalmail.pt

Correspondência: Serviço de Pneumologia - Hospital de São João, Alameda Prof. Hernâni Monteiro, 4202-451 – Porto – Portugal.
, Miguel Barbosa2, Conceição Souto Moura3, João Almeida4, Silva Ferreira5, Doutor Venceslau Hespanhol6, Doutora Fátima Carneiro7, Margarida Damasceno8
1 Interna Complementar de Pneumologia
2 Interno Complementar de Oncologia Médica
3 Assistente Hospitalar de Anatomia Patológica
4 Chefe de Serviço de Pneumologia
5 Chefe de Serviço de Medicina Interna
6 Serviço de Pneumologia Hospital de São João, Faculdade de Medicina da Universidade do Porto – Porto
7 Serviço de Anatomia Patológica Hospital de São João, Faculdade de Medicina da Universidade do Porto – Porto
8 Serviço de Oncologia Médica Hospital de São João, Faculdade de Medicina da Universidade do Porto – Porto
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Abstract

The 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 imagiological worsening and then therapy with interferon α-2a was started. Even with imagiological stability of pulmonary lesions the patient remained symptomatic and died nine months after the diagnosis had been established.

The authors emphasise the rarity of this type of pulmonary neoplasm and discuss its clinical presentation, histological features, treatment and prognosis.

Key-words:
Epithelioid hemangioendothelioma
lung
interferon α
Resumo

Os 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 a doente ter apresentado agravamento clínico e imagiológico foi iniciada terapêutica com interferão α-2a. Apesar da estabilidade imagiológica das lesões pulmonares, a doente manteve-se sintomática e faleceu nove meses depois do diagnóstico ter sido estabelecido.

Os autores realçam a raridade deste tipo de neoplasia pulmonar e discutem a sua apresentação clínica, características histológicas, tratamento e prognóstico.

Palavras-chave:
Hemangioendotelioma epitelióide
pulmão
interferão α
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Bibliography
[1.]
R. Díaz, Á. Segura, V. Calderero, et al.
Central nervous system metastases of a pulmonary epithelioid hemangioendothelioma.
Eur Respir J, 23 (2004), pp. 483-486
[2.]
P. Cronin, D. Arenberg.
Pulmonary epithelioid hemangioendothelioma – an unusual case and a review of the literature.
Chest, 125 (2004), pp. 789-792
[3.]
M. Kitaichi, S. Nagai, K. Nishimura, et al.
Pulmonary epithelioid hemangioendothelioma in 21 patients, including three with partial spontaneous regression.
Eur Respir J, 12 (1998), pp. 89-96
[4.]
C. Pinet, A. Magnan, L. Garbe, et al.
Aggressive form of pleural epithelioid hemangioendothelioma: complete response after chemotherapy.
Eur Respir J, 14 (1999), pp. 237-238
[5.]
Pankaj Kumar, I. Judson, A.G. Nicholson, et al.
Mediastinal hemangioma: successful treatment by alpha–2a interferon and postchemotherapy resection.
J Thorac Cardiovasc Surg, 124 (2002), pp. 404-406
Copyright © 2008. Sociedade Portuguesa de Pneumologia
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