Arvind Dasari1, Kathan Mehta2, Lauren A Byers3, Halfdan Sorbye4,5, James C Yao1. 1. Department of Gastrointestinal Medical Oncology, The University of Texas MD Anderson Cancer Center, Houston, Texas. 2. Department of Medicine, University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania. 3. Department of Thoracic/Head and Neck Medical Oncology, The University of Texas MD Anderson Cancer Center, Houston, Texas. 4. Department of Oncology, Haukeland University Hospital, Bergen, Norway. 5. Department of Clinical Science, University of Bergen, Bergen, Norway.
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.
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.
Authors: William D Travis; Elisabeth Brambilla; Andrew G Nicholson; Yasushi Yatabe; John H M Austin; Mary Beth Beasley; Lucian R Chirieac; Sanja Dacic; Edwina Duhig; Douglas B Flieder; Kim Geisinger; Fred R Hirsch; Yuichi Ishikawa; Keith M Kerr; Masayuki Noguchi; Giuseppe Pelosi; Charles A Powell; Ming Sound Tsao; Ignacio Wistuba Journal: J Thorac Oncol Date: 2015-09 Impact factor: 15.609
Authors: Matthew H Kulke; Manisha H Shah; Al B Benson; Emily Bergsland; Jordan D Berlin; Lawrence S Blaszkowsky; Lyska Emerson; Paul F Engstrom; Paul Fanta; Thomas Giordano; Whitney S Goldner; Thorvardur R Halfdanarson; Martin J Heslin; Fouad Kandeel; Pamela L Kunz; Boris W Kuvshinoff; Christopher Lieu; Jeffrey F Moley; Gitonga Munene; Venu G Pillarisetty; Leonard Saltz; Julie Ann Sosa; Jonathan R Strosberg; Jean-Nicolas Vauthey; Christopher Wolfgang; James C Yao; Jennifer Burns; Deborah Freedman-Cass Journal: J Natl Compr Canc Netw Date: 2015-01 Impact factor: 11.908
Authors: Pamela L Kunz; Diane Reidy-Lagunes; Lowell B Anthony; Erin M Bertino; Kari Brendtro; Jennifer A Chan; Herbert Chen; Robert T Jensen; Michelle Kang Kim; David S Klimstra; Matthew H Kulke; Eric H Liu; David C Metz; Alexandria T Phan; Rebecca S Sippel; Jonathan R Strosberg; James C Yao Journal: Pancreas Date: 2013-05 Impact factor: 3.327
Authors: Bruno Conte; Ben George; Michael Overman; Jeannelyn Estrella; Zhi-Qin Jiang; Amir Mehrvarz Sarshekeh; Renata Ferrarotto; Paulo M Hoff; Asif Rashid; James C Yao; Scott Kopetz; Arvind Dasari Journal: Clin Colorectal Cancer Date: 2015-12-29 Impact factor: 4.481
Authors: Job van Riet; Harmen J G van de Werken; Edwin Cuppen; Ferry A L M Eskens; Margot Tesselaar; Linde M van Veenendaal; Heinz-Josef Klümpen; Marcus W Dercksen; Gerlof D Valk; Martijn P Lolkema; Stefan Sleijfer; Bianca Mostert Journal: Nat Commun Date: 2021-07-29 Impact factor: 14.919
Authors: Justin P Norton; Arnaud Augert; Emily Eastwood; Ryan Basom; Charles M Rudin; David MacPherson Journal: Genes Dev Date: 2021-05-20 Impact factor: 11.361