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        "resumen" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Os tumores neuroend&#243;crinos prim&#225;rios representam cerca de 4&#37; do total dos tumores do mediastino anterior&#44; incluindo o timo&#44; afectando mais homens que mulheres numa raz&#227;o de 3&#58;1 e apresentando uma maior incid&#234;ncia entre os 40 e os 60 anos&#46; Antigamente classificados como timomas&#44; desde 1972 s&#227;o considerados um grupo distinto de neoplasias t&#237;micas&#44; podendo ser biologicamente activos&#44; estando sobretudo associados &#224; s&#237;ndroma NEM-1 &#40;19 a 25&#37; dos doentes&#44; sendo nestes casos mais agressivos&#41;&#46;</p><p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">S&#227;o descritos como tendo grande progress&#227;o local&#44; recorr&#234;ncia e met&#225;stases em elevada percentagem de casos&#44; o que determina um mau progn&#243;stico&#46; O estadiamento &#233; o par&#226;metro mais importante para determinar a sobrevida&#46; Tumores detectados em fase precoce e capsulados associam-se a um excelente progn&#243;stico&#44; enquanto em est&#225;dios avan&#231;ados&#44; localmente invasivos&#44; t&#234;m progn&#243;stico relativamente pobre&#46;</p><p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">A remo&#231;&#227;o cir&#250;rgica completa&#44; para todos os tumores t&#237;micos&#44; &#233; a base da terap&#234;utica e o factor cr&#237;tico para a sobreviv&#234;ncia a longo prazo&#44; independentemente do tipo histol&#243;gico&#46; S&#227;o uma causa de morte importante&#44; nomeadamente os tumores associados a NEM-1 e s&#237;ndroma de Cushing&#44; e h&#225; autores que defendem a realiza&#231;&#227;o de timectomia profil&#225;ctica nestes doentes&#46;</p>"
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Vol. 16. Issue 1.
Pages 177-185 (January - February 2010)
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Vol. 16. Issue 1.
Pages 177-185 (January - February 2010)
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Tumor carcinóide do timo – Caso clínico
Thymic carcinoid – case report
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Lina Carvalho1, Luís Eugénio Fernandes2, Filipe Ferreira3, Filipa Januário3, Diogo Robles3, Inês Sanches3, Edgar Vaz3, Manuel Antunes2
1 Anatomia Patológica
2 Cirurgia Cardiotorácica
3 Alunos da disciplina de Oncologia – 6.° ano – Medicina – Faculdade de Medicina da Universidade de Coimbra/6th year Oncology medical students
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Resumo

Os tumores neuroendócrinos primários representam cerca de 4% do total dos tumores do mediastino anterior, incluindo o timo, afectando mais homens que mulheres numa razão de 3:1 e apresentando uma maior incidência entre os 40 e os 60 anos. Antigamente classificados como timomas, desde 1972 são considerados um grupo distinto de neoplasias tímicas, podendo ser biologicamente activos, estando sobretudo associados à síndroma NEM-1 (19 a 25% dos doentes, sendo nestes casos mais agressivos).

São descritos como tendo grande progressão local, recorrência e metástases em elevada percentagem de casos, o que determina um mau prognóstico. O estadiamento é o parâmetro mais importante para determinar a sobrevida. Tumores detectados em fase precoce e capsulados associam-se a um excelente prognóstico, enquanto em estádios avançados, localmente invasivos, têm prognóstico relativamente pobre.

A remoção cirúrgica completa, para todos os tumores tímicos, é a base da terapêutica e o factor crítico para a sobrevivência a longo prazo, independentemente do tipo histológico. São uma causa de morte importante, nomeadamente os tumores associados a NEM-1 e síndroma de Cushing, e há autores que defendem a realização de timectomia profiláctica nestes doentes.

Palavras-chave:
Timo
carcinóide
prognóstico
Abstract

Primary neuroendocrine tumours (carcinoid tumours) account for about 4% of anterior mediastinal tumours concerning thymus localization. They appear to have a male predilection (3:1 ratio) and occur mostly between 40 and 60 years of age. Classified primarily as thymomas, they have been considered a different group of thymic neoplasia since 1972. They can be biologically active, mostly associated with MEN-1 (19–25% of patients and more aggressive in these cases).

As a locally invasive disease, with recurrence and metastasis in a high percentage of cases, it correlates with a poor prognosis. Staging is the most important determinant of survival. Encapsulated tumours diagnosed in early stages have an excellent prognosis, while locally invasive tumours in more advanced stages have a relatively poor prognosis as happens with thymomas.

Complete surgical removal is the critical factor for long-term survival rates and the basis for treatment of all thymic tumours, independent of histologic type. As an important cause of death, especially in carcinoid tumours associated with MEN-1 and Cushing's syndrome, some authors advocate the need for profilactic thymectomy in these patients.

Key-words:
Thymus
carcinoid
prognosis
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Copyright © 2010. Sociedade Portuguesa de Pneumologia
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