title: Clinical features of large cell neuroendocrine carcinoma: a population-based overview.
abstract: 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.
authors.affiliation: Dept of Pulmonary Diseases, GROW School for Oncology and Developmental Biology, Maastricht University Medical Centre, Maastricht, The Netherlands.
authors.forename: Jules L
authors.initials: JL
authors.lastname: Derks
authors.affiliation: Dept of Pulmonary Diseases, GROW School for Oncology and Developmental Biology, Maastricht University Medical Centre, Maastricht, The Netherlands.
authors.forename: Lizza E
authors.initials: LE
authors.lastname: Hendriks
authors.affiliation: Dept of Thorax Oncology, Netherlands Cancer Institute, Amsterdam, The Netherlands.
authors.forename: Wieneke A
authors.initials: WA
authors.lastname: Buikhuisen
authors.affiliation: Dept of Pulmonary Diseases, University of Groningen and University Medical Centre, Groningen, The Netherlands.
authors.forename: Harry J M
authors.initials: HJ
authors.lastname: Groen
authors.affiliation: Dept of Pathology, VU University Medical Centre, Amsterdam, The Netherlands.
authors.forename: Erik
authors.initials: E
authors.lastname: Thunnissen
authors.affiliation: Dept of Pathology, Jeroen Bosch Hospital, s' Hertogenbosch, The Netherlands.
authors.forename: Robert-Jan
authors.initials: RJ
authors.lastname: van Suylen
authors.affiliation: Dept of Radiation Oncology, MAASTRO Clinic, Maastricht, The Netherlands.
authors.forename: Ruud
authors.initials: R
authors.lastname: Houben
authors.affiliation: Dept of Registry and Research, Comprehensive Cancer Centre, Rotterdam, The Netherlands.
authors.forename: Ronald A
authors.initials: RA
authors.lastname: Damhuis
authors.affiliation: Dept of Pathology, GROW School for Oncology and Developmental Biology, Maastricht University Medical Centre, Maastricht, The Netherlands.
authors.forename: Ernst J M
authors.initials: EJ
authors.lastname: Speel
authors.affiliation: Dept of Pulmonary Diseases, GROW School for Oncology and Developmental Biology, Maastricht University Medical Centre, Maastricht, The Netherlands a.dingemans@mumc.nl.
authors.forename: Anne-Marie C
authors.initials: AM
authors.lastname: Dingemans
mesh_terms: D000230:Adenocarcinoma / Q000401:mortality / Q000473:pathology; D000368:Aged; D000369:Aged, 80 and over; D018287:Carcinoma, Large Cell / Q000401:mortality / Q000473:pathology*; D018278:Carcinoma, Neuroendocrine / Q000401:mortality / Q000473:pathology*; D002294:Carcinoma, Squamous Cell / Q000401:mortality / Q000473:pathology; D005260:Female; D006801:Humans; D008175:Lung Neoplasms / Q000401:mortality / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D009362:Neoplasm Metastasis; D009367:Neoplasm Staging; D009426:Netherlands; D016016:Proportional Hazards Models; D012042:Registries; D012189:Retrospective Studies; D055752:Small Cell Lung Carcinoma / Q000401:mortality / Q000473:pathology