Comparative study of lung and extrapulmonary poorly differentiated neuroendocrine carcinomas: A SEER database analysis of 162,983 cases

BACKGROUND Extrapulmonary neuroendocrine carcinomas (NECs) are poorly studied and are managed similar to lung NECs, which may not account for differences between the 2 groups of tumors as well as the heterogeneity within extrapulmonary NEC. METHODS Data from the Surveillance, Epidemiology, and End R...

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Bibliographic Details
Published in:Cancer 2018-02, Vol.124 (4), p.807-815
Main Authors: Dasari, Arvind, Mehta, Kathan, Byers, Lauren A., Sorbye, Halfdan, Yao, James C.
Format: Article
Language:English
Subjects:
and End Results (SEER)
Cancer
Carcinoma
Cell morphology
Comparative studies
Cytology
Epidemiology
Esophagus
Genital tract
Incidence
large cell
Lung cancer
Lungs
Medical prognosis
Morphology
neuroendocrine carcinoma (NEC)
Neuroendocrine tumors
Oncology
Pancreas
Patients
poorly differentiated
small cell
Small intestine
Statistical analysis
Subgroups
Surveillance
Survival
Tumors
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Summary:BACKGROUND Extrapulmonary neuroendocrine carcinomas (NECs) are poorly studied and are managed similar to lung NECs, which may not account for differences between the 2 groups of tumors as well as the heterogeneity within extrapulmonary NEC. METHODS Data from the Surveillance, Epidemiology, and End Results program between 1973 and 2012 were used to estimate the relative percentages of lung NECs and subgroups of extrapulmonary NECs, epidemiological patterns at these sites, and the median and 5‐year overall survival rates. RESULTS Of 162,983 NEC cases, 14,732 were extrapulmonary; of these, 5509 were gastrointestinal (37.44%), 4151 were of unknown primary (28.2%), and 5072 were of other sites (34.4%). Lung NEC had the highest percentage of small cell morphology (95.2%) and gastrointestinal NEC had the least (38.7%), with the rest being other morphologies. Significant differences were noted with regard to median age (range, 48‐74 years), percentage of cases of distant stage disease (24%‐77%), and incidence according to sex and race. The median survival of patients with lung NEC was 7.6 months, that for patients with gastrointestinal NEC was 7.5 months (range, 25.1 months for NEC at the small intestine to 5.7 months for NEC at the pancreas), and that for patients with unknown NEC was 2.5 months. The 5‐year survival rate for patients with local stage disease ranged from 58% to 60% for NECs of the female genital tract and small intestine to 25% for esophageal NECs. The primary tumor site remained statistically significant for survival even after adjusting for known prognostic variables (P
ISSN:0008-543X
1097-0142
DOI:10.1002/cncr.31124