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

Abstract

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.

Unmet Needs in High-Grade Gastroenteropancreatic Neuroendocrine Neoplasms (WHO G3)

“…NEC encompasses 2 pathological entities: small-cell and large-cell carcinoma. There is a small-cell histological preponderance in the squamous cell parts (esophagus and anus) and a large-cell carcinoma in the glandular parts [14]. Small-cell carcinoma was the first category described in both the lungs and the GEP tract, and for this reason most of the published literature is focused on small-cell carcinoma.…”

“…NEC can originate anywhere in the GEP tract, but they are mainly located in the esophagus, stomach, pancreas, and large bowel [7,[11][12][13][14]. Given their aggressive nature, most patients have metastatic disease at the time of presentation [5,7,[13][14][15]. In general, high-quality epidemiological data on NEC are lacking, especially as data on differentiation and proliferation rates to ensure a correct diagnosis are often not available in registries.…”

“…There has been an increase in the incidence of GEP NEC over the last 2 decades, although more awareness of this entity among pathologists could partly explain this increase [3,[8][9][10]. NEC can originate anywhere in the GEP tract, but they are mainly located in the esophagus, stomach, pancreas, and large bowel [7,[11][12][13][14]. Given their aggressive nature, most patients have metastatic disease at the time of presentation [5,7,[13][14][15].…”

“…In general, high-quality epidemiological data on NEC are lacking, especially as data on differentiation and proliferation rates to ensure a correct diagnosis are often not available in registries. In the SEER database the median survival is 34 months with localized disease, 14-16 months with regional disease, and 5 months with distant disease, but it varies by primary site [4,14]. Long-term relapse-free survival is possible among NEC patients with localized disease and seems to depend on the primary site location, but inaccurate TNM classification precludes firm conclusions [14,16].…”

“…In the SEER database the median survival is 34 months with localized disease, 14-16 months with regional disease, and 5 months with distant disease, but it varies by primary site [4,14]. Long-term relapse-free survival is possible among NEC patients with localized disease and seems to depend on the primary site location, but inaccurate TNM classification precludes firm conclusions [14,16]. The mean survival in GEP NEC patients with metastatic disease treated with chemotherapy is 11-12 months [5,7,11,12].…”