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Objectives: Pulmonary atypical carcinoids are rare tumors and controversy exists regarding management and prognosis. They have distinct clinicopathological features than typical carcinoids. Atypical carcinoids behave more aggressively compared to typical carcinoids. The purpose of this retrospective study was to describe characteristics, and outcomes of patients surgically treated for pulmonary atypical carcinoid tumors.
Methods: All patients with a diagnosis of primary pulmonary atypical carcinoid tumor surgically treated from 2014 to 2018 were reviewed. Data collected included demographics, tobacco use, clinical presentation, tumor size and location, pathological stage, immunohistochemical features, lymph node status, treatment, and survival.
Results: There were 18 male and 17 female patients treated surgically for atypical carcinoid with a mean age of 47.3 years (19-72 years). Symptoms were present in 28 patients (80%). Twenty-three patients (65.7%) had a centrally located tumor and 12 patients (34.2%) presented with peripheral tumor localization. Operations included 27 lobectomies, 4 pneumonectomies, 1 bilobectomies, 2 sleeve lobectomies, and 1 wedge resection and partial rib resection. There were thirteen patients in stage IA, five in stage IB, three in stage IIA, eight in stage IIB, one in stage IIIA and five in stage IIIB. One (2.8%) patient died and one (2.8%) patient relapsed during the follow-up period. The pathology of the relapsed patient was multiple atypical carcinoid tumor and 4 years later metastasis developed in the liver. The tumor size of the dead patient was 7 cm, the patient underwent left pneumonectomy and N2 and N1 lymph nodes were positive. He died of cardiac causes 45 months after the operation. The mean follow-up was 2.5 years.
Conclusion: In our study, we noticed stage of the disease and the size of surgical resection to be prognostic factors of atypical carcinoid tumors. Atypical carcinoid tumors tend to be more metastatic and had worse prognosis when compared with typical carcinoid tumors. We conclude that surgery is the best treatment of choice for atypical carcinoid tumors, especially parenchyma-sparing procedures, because of their good mid- and long-term survival rates. Lymph-nodes involvement is the major adverse prognostic factor in atypical carcinoids.