Clinical features of large cell neuroendocrine carcinoma: a population-based overview
Clinical features of large cell neuroendocrine carcinoma: a population-based overview
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DOI：
10.1183/13993003.00618-2015
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发表时间：
2016-02-01
影响因子：
24.3
通讯作者：
Dingemans, Anne-Marie C.
中科院分区：
文献类型：
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作者：
Derks, Jules L.;Hendriks, Lizza E.;Dingemans, Anne-Marie C.
肺大细胞神经内分泌癌（LCNEC）是一种罕见疾病，关于其临床特征的数据很少。因此，我们分析了在荷兰癌症登记处登记的LCNEC，并将数据与小细胞肺癌（SCLC）、鳞状细胞癌（SqCC）和腺癌（AdC）进行比较，从荷兰癌症登记处（2003-2012）中选择组织学证实的LCNEC（n=952）、SCLC（n=11844）、SqCC（n=19633）和AdC（n=24253）病例。比较了I-II、III和IV期疾病的患者特征、诊断时的转移（2006年或以后）、包括多变量考克斯模型和一线治疗的总生存（OS）。LCNEC病例数从2003年的56例增加到2012年的143例，占所有肺癌的0.9%。IV期LCNEC患者（n=383）通常在肝脏（47%）、骨（32%）和脑（23%）中发生转移，类似于SCLC。I-II期、III期和IV期LCNEC患者的中位OS（95% CI）分别为32.4（22.0-42.9）、12.6（10.3-15.0）和4.0（3.5-4.6）个月。LCNEC患者的多变量校正OS与SCLC患者相似，但低于SqCC和AdC患者。然而，手术切除和辅助化疗的频率更像SqCC和AdC而不是SCLC。LCNEC的转移模式类似于小细胞肺癌，OS也是如此。然而，早期治疗策略似乎与SqCC和AdC更具可比性。
Pulmonary large cell neuroendocrine carcinoma (LCNEC) is an orphan disease and few data are available on its clinical characteristics. Therefore, we analysed LCNEC registered in the Netherlands Cancer Registry, and compared data with small cell lung carcinoma (SCLC), squamous cell carcinoma (SqCC) and adenocarcinoma (AdC).Histologically confirmed LCNEC (n=952), SCLC (n=11844), SqCC (n=19633) and AdC (n=24253) cases were selected from the Netherlands Cancer Registry (2003-2012). Patient characteristics, metastasis at diagnosis (2006 or later), overall survival (OS) including multivariate Cox models and first-line treatment were compared for stage I-II, III and IV disease.The number of LCNEC cases increased from 56 patients in 2003 to 143 in 2012, accounting for 0.9% of all lung cancers. Stage IV LCNEC patients (n=383) commonly had metastasis in the liver (47%), bone (32%) and brain (23%), resembling SCLC. Median OS (95% CI) of stage I-II, III and IV LCNEC patients was 32.4 (22.0-42.9), 12.6 (10.3-15.0) and 4.0 (3.5-4.6) months, respectively. Multivariate-adjusted OS of LCNEC patients resembled that of SCLC patients, and was poorer than those of SqCC and AdC patients. However, frequency of surgical resection and adjuvant chemotherapy resembled SqCC and AdC more than SCLC.Diagnosis of LCNEC has increased in recent years. The metastatic pattern of LCNEC resembles SCLC as does the OS. However, early-stage treatment strategies seem more comparable to those of SqCC and AdC.