Clinical features of large cell neuroendocrine carcinoma: A population-based overview

Jules L. Derks, Lizza E. Hendriks, Wieneke A. Buikhuisen, Harry J.M. Groen, Erik Thunnissen, Robert Jan Van Suylen, Ruud Houben, Ronald A. Damhuis, Ernst J.M. Speel, Anne Marie C. Dingemans*

*Corresponding author for this work

Research output: Contribution to journalArticleAcademicpeer-review

97 Citations (Scopus)


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=11 844), SqCC (n=19 633) and AdC (n=24 253) 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.

Original languageEnglish
Pages (from-to)615-624
Number of pages10
JournalEuropean Respiratory Journal
Issue number2
Publication statusPublished - 1 Feb 2016
Externally publishedYes

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Publisher Copyright:
Copyright © 2016 by the European Respiratory Society.


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