Abstract

Despite rising incidence, there remains limited data guiding the prognostication and treatment of patients with bronchopulmonary carcinoid tumors, particularly atypical carcinoids. We report outcomes of a large, modern, single-institutional series of patients treated for localized or locally advanced atypical carcinoid of the lung. We retrospectively analyzed the demographic, histologic and treatment histories of 69 patients (74% female) with median age of 65 at diagnosis (range 31-83) who were treated between 2004-2016. The Kaplan-Meier method was used for overall survival (OS) estimates and compared by log-rank. Cox proportional hazards models were used for univariate (UVA) and multivariate analyzes (MVA). Median follow-up time was 33.6 months. The majority (96%) of patients underwent surgical resection (86% R0, 9% R1, 3% R2) with common approaches being lobectomy (59%), wedge resection (13%) and pneumonectomy (9%). Three patients (4%) received definitive radiotherapy as their local treatment. Nearly half (49%) of patients had nodal involvement with a stage distribution of 39% stage I, 25% stage II and 36% stage III. Twenty-one patients received chemotherapy as part of their initial treatment, 81% of whom had stage III disease. Sixteen patients received radiotherapy (median 50.4 Gy, range 18-66 Gy) as part of their initial treatment, most of whom received postoperative radiation for N2 disease (63%). Five patients (31%) received postoperative radiotherapy due to concern of incompletely resected disease. Higher stage was significantly associated with poorer OS (p=0.04). 3-year OS for Stage I, II and III disease was 96%, 88% and 72%, respectively. Stage I disease also had a significantly lower risk of distant metastasis compared to Stage II/III disease (17% vs. 31% at 3 years p=0.04). On UVA, Stage III disease was significantly associated with poorer OS (HR 4.7, p=0.021) and risk of distant failure (HR 2.8, p=0.039). Multivariate modeling showed that older age (HR 1.05, p=0.03) and stage III status (HR 6.6, p=0.009) were predictive of poorer OS. For stage III patients treated surgically, receipt of adjuvant therapy (chemotherapy and/or radiotherapy) was not significantly associated with OS (p=0.36) or distant failure (p=0.69). This is one of the largest reported series of atypical pulmonary carcinoid patients treated with curative intent. We observed generally favorable prognosis in this cohort that was primarily treated with surgery. We did not observe a significant impact of adjuvant therapy on outcomes, but small patient numbers limit our ability to quantify their potential effect.

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