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Background  Pulmonary large-cell neuroendocrine carcinoma (pLCNEC) is a very rare malignancy originating from the lung and bronchus, and its biological behaviour, clinical diagnosis, treatment and prognosis are poorly understood. Thus, the clinical characteristics and surgical treatment-related prognostic factors of this rare disorder must be explored.    Results  The clinical data of 59 patients (48 males and 11 females) who were treated by surgery and diagnosed with pLCNEC by postoperative pathology at Peking Union Medical College Hospital from April 2004 to April 2019 were analysed retrospectively. The median patient age was 62 years (38–79 years), and the median duration of disease was 2 months (0.5–18 months). Compared with other lung malignancies, pLCNEC lacks specific clinical symptoms and imaging features, and preoperative biopsy pathology is often insufficient to confirm the diagnosis. The corresponding numbers of patients who were classified into stages I, II, III and IV according to the postoperative pathological tumour-nodal-metastasis stage were 25, 12, 15 and 7, respectively. The median overall survival was 36 months (0.9–61.1 months). The 1-year, 3-year and 5-year survival rates were 76.3%, 49% and 44.7%, respectively. The tumour stage exerted a significant effect on survival (Cox multivariate analysis p < 0.05).    Conclusions  For patients with resectable pLCNEC, multidisciplinary therapy based on surgery may have good survival benefits, and tumour stage is an independent risk factor for the prognosis of pLCNEC.

orphanet journal of rare diseases

Survival outcomes of surgery in patients with pulmonary large-cell neuroendocrine carcinoma: a retrospective single-institution analysis and literature review