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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)
Caso Clínico/Case Report
Open Access
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.º 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 (19a 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.

Rev Port Pneumol 2010; XVI (1): 177-185

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.

Rev Port Pneumol 2010; XVI (1): 177-185

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