Department of Thoracic Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, 100730, China.
Orphanet J Rare Dis. 2021 Feb 12;16(1):82. doi: 10.1186/s13023-021-01730-7.
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.
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).
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.
肺大细胞神经内分泌癌（pLCNEC）是一种非常罕见的起源于肺和支气管的恶性肿瘤，其生物学行为、临床诊断、治疗和预后尚不清楚。因此，必须探讨这种罕见疾病的临床特征和与手术治疗相关的预后因素。
回顾性分析了 2004 年 4 月至 2019 年 4 月北京协和医学院医院因手术治疗和术后病理诊断为 pLCNEC 的 59 例患者（48 例男性和 11 例女性）的临床资料。患者中位年龄为 62 岁（38-79 岁），中位疾病持续时间为 2 个月（0.5-18 个月）。与其他肺部恶性肿瘤相比，pLCNEC 缺乏特异性的临床症状和影像学特征，术前活检病理往往不足以明确诊断。根据术后病理肿瘤-淋巴结-转移分期，分别有 25 例、12 例、15 例和 7 例患者被归类为Ⅰ期、Ⅱ期、Ⅲ期和Ⅳ期。中位总生存期为 36 个月（0.9-61.1 个月）。1 年、3 年和 5 年生存率分别为 76.3%、49%和 44.7%。肿瘤分期对生存有显著影响（Cox 多因素分析 p<0.05）。
对于可切除的 pLCNEC 患者，基于手术的多学科治疗可能具有良好的生存获益，肿瘤分期是影响 pLCNEC 预后的独立危险因素。
