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Vol. 16. Issue 3.
Pages 483-492 (May - June 2010)
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Vol. 16. Issue 3.
Pages 483-492 (May - June 2010)
Caso Clínico/Case Report
Open Access
Tumor maligno da bainha dos nervos periféricos do pulmão: A propósito de um caso clínico
Malignant peripheral nerve sheath tumors: A case report
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N. Serrano Marçal1, E. Teixeira2, R. Sotto-Mayor3, A. Manique4, P. Campos5, J. Cruz6, M. Mendes de Almeida7, A. Bugalho de Almeida8
1 Interno do internato complementar de Pneumologia*
2 Assistente hospitalar graduada de Pneumologia*
3 Chefe de serviço de Pneumologia*
4 Assistente hospitalar graduada de Pneumologia*
5 Assistente hospitalar graduada de Radiologia
6 Assistente hospitalar graduado de Cirurgia Cardiotorácica
7 Assistente hospitalar graduada de Anatomia Patológica§
8 Chefe de serviço de Pneumologia*
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Resumo

Os tumores malignos da bainha dos nervos periféricos correspondem a um grupo raro de sarcomas de tecidos moles que tendem a ocorrer em doentes com neurofibromatose tipo 1 ou vários anos após tratamentos de radioterapia. A sua localização torácica é uma entidade muito rara. A sintomatologia habitual deve-se ao efeito de massa exercido sobre os nervos, podendo persistir durante meses ou anos antes do diagnóstico. A escassez de doentes com este tipo de tumor faz com que a abordagem terapêutica ainda permaneça em debate, sendo a cirurgia o procedimento de eleiçáo. O prognóstico destes doentes é mau dada a grande recorrência local e metastização. O caso clínico descrito serve de tema à discussão.

Rev Port Pneumol 2010; XVI (3): 483-492

Palavras-chave:
Tumor maligno
bainha nervo periférico
Abstract

Malignant peripheral nerve sheath tumors comprehend a rare group of soft tissue sarcomas that tend to occur in patients with neurofibromatosis type 1 or several years after radiotherapy treatments. Its thoracic localization is a very unusual entity. The typical symptoms are due to nerve roots compression which can persist for several months or years before diagnosis. Due to very few patients with this type of tumor its therapeutic approach is still a matter of permanent debate, being surgery the main treatment. This tumor has a bad prognosis because of high local recurrence and metastasis. The clinical case we describe serves as a glimpse for discussion.

Rev Port Pneumol 2010; XVI (3): 483-492

Key-words:
Malignant tumour
peripheral nerve sheath
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