Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Comparative Study
. 2018 Feb 15;124(4):807-815.
doi: 10.1002/cncr.31124. Epub 2017 Dec 6.

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

Arvind Dasari  1 , Kathan Mehta  2 , Lauren A Byers  3 , Halfdan Sorbye  4   5 , James C Yao  1
Affiliations
Comparative Study

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

Arvind Dasari et al. Cancer. .

Abstract

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<.0001).

Conclusions: To the authors' knowledge, the current study is the largest study of NECs performed to date and also the first with comprehensive epidemiological data. Significant differences in incidence patterns and large variations in survival depending on anatomical site and morphological subtype were noted. A curative approach is possible for patients with nonmetastatic NECs. Cancer 2018;124:807-15. © 2017 American Cancer Society.

Keywords: Epidemiology; Surveillance; and End Results (SEER); large cell; neuroendocrine carcinoma (NEC); poorly differentiated; small cell.

PubMed Disclaimer

Conflict of interest statement

Disclosures

The authors have declared no conflicts of interest

Figures

Figure 1
Figure 1
Proportion of poorly differentiated neuroendocrine carcinomas according to morphological subtype at major sites (1A), subsites including non-pulmonary, non-gastrointestinal (Other) sites (1B), gastrointestinal tract (1C).
Figure 1
Figure 1
Proportion of poorly differentiated neuroendocrine carcinomas according to morphological subtype at major sites (1A), subsites including non-pulmonary, non-gastrointestinal (Other) sites (1B), gastrointestinal tract (1C).
Figure 1
Figure 1
Proportion of poorly differentiated neuroendocrine carcinomas according to morphological subtype at major sites (1A), subsites including non-pulmonary, non-gastrointestinal (Other) sites (1B), gastrointestinal tract (1C).
Figure 1
Figure 1
Proportion of poorly differentiated neuroendocrine carcinomas according to morphological subtype at major sites (1A), subsites including non-pulmonary, non-gastrointestinal (Other) sites (1B), gastrointestinal tract (1C).
Figure 1
Figure 1
Proportion of poorly differentiated neuroendocrine carcinomas according to morphological subtype at major sites (1A), subsites including non-pulmonary, non-gastrointestinal (Other) sites (1B), gastrointestinal tract (1C).
Figure 1
Figure 1
Proportion of poorly differentiated neuroendocrine carcinomas according to morphological subtype at major sites (1A), subsites including non-pulmonary, non-gastrointestinal (Other) sites (1B), gastrointestinal tract (1C).
Figure 2
Figure 2
Proportion of poorly differentiated neuroendocrine carcinomas according to morphological subtype at subsites including urinary system (2A), female (2B) and male (2C) genital tracts.
Figure 2
Figure 2
Proportion of poorly differentiated neuroendocrine carcinomas according to morphological subtype at subsites including urinary system (2A), female (2B) and male (2C) genital tracts.
Figure 2
Figure 2
Proportion of poorly differentiated neuroendocrine carcinomas according to morphological subtype at subsites including urinary system (2A), female (2B) and male (2C) genital tracts.
Figure 2
Figure 2
Proportion of poorly differentiated neuroendocrine carcinomas according to morphological subtype at subsites including urinary system (2A), female (2B) and male (2C) genital tracts.
Figure 2
Figure 2
Proportion of poorly differentiated neuroendocrine carcinomas according to morphological subtype at subsites including urinary system (2A), female (2B) and male (2C) genital tracts.
Figure 2
Figure 2
Proportion of poorly differentiated neuroendocrine carcinomas according to morphological subtype at subsites including urinary system (2A), female (2B) and male (2C) genital tracts.
Figure 3
Figure 3
Median (3A) and 5-year survival (3B) of poorly differentiated neuroendocrine carcinomas across primary sites according to stage. “*” : median survival not reached.
Figure 3
Figure 3
Median (3A) and 5-year survival (3B) of poorly differentiated neuroendocrine carcinomas across primary sites according to stage. “*” : median survival not reached.
See this image and copyright information in PMC

References

    1. Klimstra DS, Modlin IR, Coppola D, Lloyd RV, Suster S. The pathologic classification of neuroendocrine tumors: a review of nomenclature, grading, and staging systems. Pancreas. 2010;39(6):707–712. - PubMed
    1. Travis WD, Brambilla E, Nicholson AG, et al. The 2015 World Health Organization Classification of Lung Tumors: Impact of Genetic, Clinical and Radiologic Advances Since the 2004 Classification. J Thorac Oncol. 2015;10(9):1243–1260. - PubMed
    1. Soto DE, Eisbruch A. Limited-stage extrapulmonary small cell carcinoma: outcomes after modern chemotherapy and radiotherapy. Cancer J. 2007;13(4):243–246. - PubMed
    1. Ochsenreither S, Marnitz-Schultze S, Schneider A, et al. Extrapulmonary small cell carcinoma (EPSCC): 10 years' multi-disciplinary experience at Charite. Anticancer Res. 2009;29(8):3411–3415. - PubMed
    1. Kim JH, Lee SH, Park J, et al. Extrapulmonary small-cell carcinoma: a single-institution experience. Jpn J Clin Oncol. 2004;34(5):250–254. - PubMed

Publication types