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Os tumores neuroendócrinos do pulmão são classificados de acordo com os critérios histológicos da Organização Mundial de Saúde de 2004: carcinóide típico, carcinóide atípico, carcinoma neuroendócrino de grandes células e carcinoma pulmonar de pequenas células. A característica histológica mais importante para distinguir o tipo de tumor neuroendócrino é o índice mitótico.
Os carcinóides pulmonares são uma patologia rara. Representam aproximadamente 2% de todas as neoplasias malignas do pulmão e correspondem a 25% dos tumores neuroendócrinos de todo o corpo humano. O carcinóide típico é mais frequente, correspondendo a 80-90% dos casos.
Um terço a metade dos indivíduos são assintomáticos na altura do diagnóstico. Quando sintomáticos, apresentam tipicamente tosse, hemoptises e pneumonia obstrutiva. A tomografia computorizada do tórax com contraste é o melhor método imagiológico de diagnóstico, mas é necessário exame histológico para a sua correcta classificação. O índice proliferativo de Ki-67 não permite a distinção entre carcinóide tipíco e atípico, mas é útil na separação de tumores carcinóides e de tumores neuroendócrinos de alto grau de malignidade, carcinoma neuroendócrino de grandes células e carcinoma pulmonar de pequenas células, especialmente em biopsias de pequenas dimensões.
A ressecção cirúrgica é a primeira opção terapêutica e representa a única possibilidade de cura. O papel da radioterapia e quimioterapia é ainda incerto.
Os principais factores que influenciam o prognóstico são o subtipo histológico, envolvimento ganglionar, presença de metástases e dimensões do tumor.
O carcinóide típico apresenta uma excelente taxa de sobrevivência, com rara metastização.
Por sua vez, o carcinóide atípico, devido ao seu comportamento mais agressivo, tem um pior prognóstico.
The 2004 World Health Organization classification recognizes four major types of lung neuroendocrine tumors: typical carcinoid, atypical carcinoid, large cell neuroendocrine carcinoma and small cell lung cancer. The most important histologic feature used to distinguish the grade of lung neuroendocrine tumor is the mitotic count. Carcinoid tumors of the lung are rare. They are reported to account for approximately 25% of all carcinoid tumors and represent only 1% to 2% of all lung cancers. Typical carcinoid is more frequent, representing 80-90% of the cases. One third to half of patients are asymptomatic at diagnosis. Patients typically present: cough, hemoptysis and post-obstructive pneumonia. Thoracic computed tomography with contrast is the diagnostic gold standard for pulmonary carcinoids, but pathology examination is mandatory for their correct classification. Ki-67 is not established as a way to distinguish typical carcinoid from atypical but it is very useful for the separation of carcinoid tumors from the high-grade large cell neuroendocrine carcinoma and small cell lung cancer, particularly in small biopsies. Radical surgery intervention is the mainstay of treatment and the only curative option. The role of radiotherapy and chemotherapy is still debated. Histological subtype, nodal involvement, distant metastasis and tumor dimensions appear as the most important factors influencing the prognosis. Typical carcinoids are associated with excellent long-term survival, with rare metastatic spread. Atypical carcinoids, due to a more malignant potential, exhibit worse prognosis.
The 2004 World Health Organization classification recognizes four major types of lung neuroendocrine tumors: typical carcinoid, atypical carcinoid, large cell neuroendocrine carcinoma and small cell lung cancer. The most important histologic feature used to distinguish the grade of lung neuroendocrine tumor is the mitotic count. Carcinoid tumors of the lung are rare. They are reported to account for approximately 25% of all carcinoid tumors and represent only 1% to 2% of all lung cancers. Typical carcinoid is more frequent, representing 80-90% of the cases. One third to half of patients are asymptomatic at diagnosis. Patients typically present: cough, hemoptysis and post-obstructive pneumonia. Thoracic computed tomography with contrast is the diagnostic gold standard for pulmonary carcinoids, but pathology examination is mandatory for their correct classification. Ki-67 is not established as a way to distinguish typical carcinoid from atypical but it is very useful for the separation of carcinoid tumors from the high-grade large cell neuroendocrine carcinoma and small cell lung cancer, particularly in small biopsies. Radical surgery intervention is the mainstay of treatment and the only curative option. The role of radiotherapy and chemotherapy is still debated. Histological subtype, nodal involvement, distant metastasis and tumor dimensions appear as the most important factors influencing the prognosis. Typical carcinoids are associated with excellent long-term survival, with rare metastatic spread. Atypical carcinoids, due to a more malignant potential, exhibit worse prognosis.
Descrição
Trabalho Final do Curso de Mestrado Integrado em Medicina, Faculdade de Medicina, Universidade de Lisboa, 2015
Palavras-chave
Tumor carcinóide Pulmão Neoplasias pulmonares Pneumologia
