Tumor maligno da bainha dos nervos periféricos do pulmão: A propósito de um caso clínico
Malignant peripheral nerve sheath tumors: A case report
N. Serrano Marçal1,
, E. Teixeira2, R. Sotto-Mayor3, A. Manique4, P. Campos5, J. Cruz6, M. Mendes de Almeida7, A. Bugalho de Almeida8
Corresponding author
1 Interno do internato complementar de Pneumologia, Serviço de Pneumologia 1, Hospital de Santa Maria, CHLN, EPE, Lisboa
2 Assistente hospitalar graduada de Pneumologia, Serviço de Pneumologia 1, Hospital de Santa Maria, CHLN, EPE, Lisboa
3 Chefe de serviço de Pneumologia, Serviço de Pneumologia 1, Hospital de Santa Maria, CHLN, EPE, Lisboa
4 Assistente hospitalar graduada de Pneumologia, Serviço de Pneumologia 1, Hospital de Santa Maria, CHLN, EPE, Lisboa
5 Assistente hospitalar graduada de Radiologia, Serviço de Imagiologia Geral 1, Hospital de Santa Maria, CHLN, EPE, Lisboa
6 Assistente hospitalar graduado de Cirurgia Cardiotorácica, Serviço de Cirurgia Cardio-Torácica, Hospital de Santa Maria, CHLN, EPE, Lisboa
7 Assistente hospitalar graduada de Anatomia Patológica, Serviço de Anatomia Patológica, Hospital de Santa Maria, CHLN, EPE, Lisboa
8 Chefe de serviço de Pneumologia, Serviço de Pneumologia 1, Hospital de Santa Maria, CHLN, EPE, Lisboa
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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 eleição. O prognóstico destes doentes é mau dada a grande recorrência local e metastização. O caso clínico descrito serve de tema à discussão.</p>" ] "en" => array:2 [ "titulo" => "Abstract" "resumen" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">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.</p>" ] ] "bibliografia" => array:2 [ "titulo" => "Bibliografia" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:13 [ 0 => array:3 [ "identificador" => "bib1" "etiqueta" => "1." "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Malignant peripheral nerve sheath tumor: molecular pathogenesis and current management considerations" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:5 [ 0 => "S. Grobmyer" 1 => "J. Reith" 2 => "A. Shahlaee" 3 => "C. Bush" 4 => "S. 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Article information
ISSN: 21735115Original language: Portuguese
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