Background: APC is a rare thoracic malignancy with limited prognostic data and a perceived lack of treatment options other than surgical resection. This is the largest series of APC patients (pts) ever reported and contains data on locally advanced and metastatic disease. Here we compare clinical outcomes and treatment responses for typical carcinoid (TC) and atypical carcinoid (AC). Methods: This retrospective cohort includes pts referred to JH from 1992 to 2012. Pts were identified using pathology and medical record databases. Information extracted included, pathology, demographics, stage, prior curative resection, time to relapse, chemotherapy/targeted agents and response rate (RR), date of death/last follow-up. Primary outcome was overall survival (OS), defined as time from diagnosis of advanced disease to death from any cause. Secondary endpoint was recurrence-free survival (RFS), defined as time from curative resection to tumor recurrence. OS and RFS were estimated by using the Kaplan-Meier method and log-rank test comparing TC and AC. Results: 49 APC pts (30 female/19 male; 32 AC/17 TC) were identified. 25 pts had relapsed after previous curative resection. Median RFS (n=25), was significantly longer for TC vs. AC (119 months (m) vs. 66 m, p=0.008). Median OS was 85m and significantly longer for TC vs. AC (122m vs. 48m, p=0.009). 39 (80%) pts received systemic therapy for APC. RR to first-line chemotherapy was 22% (5/23) with all 5 (3 AC, 2 TC) responses occurring in pts receiving cisplatin/etoposide. First-line somatostatin therapy was given to 11 pts with 1 partial response (PR) (9%) and 10 pts with stable disease (5 AC, 5 TC) for a median 15m. 5 pts received first-line targeted agents including sunitinib and gefitinib however no responses were seen. 50% (2/4 pts) RR occurred to second-line temozolomide/capecitabine An ongoing PR > 6m to everolimus occurred in a heavily pretreated AC pt. Conclusions: Both AC and TC may recur many years after resection. This study demonstrates significantly poorer survival for AC in advanced disease. Thoracic oncologists perceive APC as resistant to systemic therapy however here we report that both AC and TC may respond to cisplatin/etoposide, temozolomide/capecitabine and mTOR targeted therapy.