Neuroendocrine Tumors Dana-Farver Cancer Institute Harvard Medical School


Bronchial Carcinoid Tumors

Bronchial carcinoids can be classified along a spectrum of pulmonary neuroendocrine tumors, of which small cell lung cancer is the most malignant. Typical carcinoids, or well-differentiated pulmonary neuroendocrine tumors are usually central in location, and symptomatic patients often present with recurrent pneumonia, cough, hemoptysis, or chest pain. These tumors may also exhibit a variety of neuroendocrine manifestations. Ectopic secretion of ACTH from bronchial carcinoid tumors accounts for 1% of all cases of Cushing’s syndrome. The carcinoid syndrome occurs in less than 5% of cases. Well-differentiated pulmonary neuroendocrine tumors are generally indolent, with metastases reported in less than 15% of cases. Metastases, when they occur, usually develop in mediastinal lymph nodes, liver, bone, or skin.

Approximately one third of bronchial carcinoids demonstrate “atypical” histologic features and are more accurately classified as well differentiated pulmonary neuroendocrine carcinomas. Atypical carcinoids occur in older individuals, most commonly in the 6th decade of life. They tend to be larger in size than well- differentiated neuroendocrine tumors and are more commonly peripheral in location. Atypical carcinoids pursue an aggressive clinical course, metastasizing to mediastinal lymph nodes in 30-50% of cases.

Conservative resection, consisting of wedge or segmental resection, is currently the preferred form of treatment for localized bronchial carcinoid tumors. Such procedures have resulted in low rates of recurrence and excellent long-term survival. The adequacy of conservative resection has been questioned in patients with atypical carcinoids, and many physicians advocate more extensive surgical procedures in this setting.

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