Incidence, patterns of care and prognostic factors for outcome of gastroenteropancreatic neuroendocrine tumors (GEP-NETs): results from the National Cancer Registry of Spain (RGETNE).
BACKGROUND
Neuroendocrine tumors (NETs) are an unusual family of neoplasms with a wide and complex spectrum of clinical behavior. Here, we present the first report of a National Cancer Registry of gastroenteropancreatic neuroendocrine tumors from a Southern European country.


METHODS
Data was provided online at www.retegep.net by participating centers and assessed for internal consistency by external independent reviewers.


RESULTS
The study cohort comprised 907 tumors. The most common tumor types were carcinoids (55%), pancreatic nonfunctional tumors (20%), metastatic NETs of unknown primary (9%), insulinomas (8%) and gastrinomas (4%). Forty-four percent presented with distant disease at diagnosis, most often those from small intestine (65%), colon (48%), rectum (40%) and pancreas (38%), being most unusual in appendix primaries (1.3%). Stage at diagnosis varied significantly according to sex, localization of primary tumor, tumor type and grade. Overall 5-year survival was 75.4% (95% confidence interval 71.3% to 79.5%) and was significantly greater in women, younger patients and patients with hormonal syndrome and early stage or lower grade tumors. Prognosis also differed according to tumor type and primary tumor site. However, stage and Ki-67 index were the only independent predictors for survival.


CONCLUSIONS
This national database reveals relevant information regarding epidemiology, current clinical practices and prognosis of NETs in Spain, providing valuable insights that may contribute to understand regional disparities in the incidence, patterns of care and survival of this heterogeneous disease across different continents and countries.
Department of Medical Oncology, Instituto de Biomedicina de Sevilla, Hospital Universitario Virgen del Rocío, Sevilla. Electronic address: rgcarbonero@hotmail.com.
R
R
Garcia-Carbonero
Department of Medical Oncology, Vall d'Hebron University Hospital, Barcelona.
J
J
Capdevila
Department of Medical Oncology, Hospital Central de Asturias, Oviedo.
G
G
Crespo-Herrero
Department of Endocrinology, Hospital Clínico Universitario San Carlos, Madrid.
J A
JA
Díaz-Pérez
Department of Medical Oncology, Hospital de Basurto, Vizcaya.
M P
MP
Martínez Del Prado
Department of Medical Oncology, Hospital Miguel Servet, Zaragoza.
V
V
Alonso Orduña
Department of Medical Oncology, Hospital Virgen de la Victoria, Málaga.
I
I
Sevilla-García
Department of Endocrinology, Hospital de Bellvitge, L'Hospitalet, Barcelona.
C
C
Villabona-Artero
Department of Medical Oncology, Hospital de Donostia, San Sebastián.
A
A
Beguiristain-Gómez
Department of Medical Oncology, Hospital Universitario de Canarias, Santa Cruz de Tenerife.
M
M
Llanos-Muñoz
Department of Endocrinology, Hospital de la Princesa.
M
M
Marazuela
Department of Endocrinology, Hospital La Paz.
C
C
Alvarez-Escola
Department of Medical Oncology, Hospital 12 de Octubre, Madrid.
D
D
Castellano
Department of Internal Medicine, University of Michigan, MI, USA.
E
E
Vilar
Department of Medical Oncology, Hospital Central de Asturias, Oviedo.
P
P
Jiménez-Fonseca
Department of Medical Oncology, Hospital Duran i Reynals, Institut Catalá d'Oncologia, Barcelona.
A
A
Teulé
Department of Medical Oncology, Hospital Clínico Universitario San Carlos, Madrid.
J
J
Sastre-Valera
Department of Medical Oncology, Instituto de Biomedicina de Sevilla, Hospital Universitario Virgen del Rocío, Sevilla.
M
M
Benavent-Viñuelas
Department of Statistics, Faculty of Biology, Barcelona University, Barcelona, Spain.
A
A
Monleon
Department of Medical Oncology, Hospital Duran i Reynals, Institut Catalá d'Oncologia, Barcelona.
R
R
Salazar
D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D002648:Child; D003695:Delivery of Health Care / Q000592:standards*; D005260:Female; D005770:Gastrointestinal Neoplasms / Q000175:diagnosis* / Q000453:epidemiology / Q000628:therapy; D006801:Humans; D015994:Incidence; D008875:Middle Aged; D009362:Neoplasm Metastasis; D009367:Neoplasm Staging; D018358:Neuroendocrine Tumors / Q000175:diagnosis* / Q000453:epidemiology / Q000628:therapy; D010190:Pancreatic Neoplasms / Q000175:diagnosis* / Q000453:epidemiology / Q000628:therapy; D011379:Prognosis; D012042:Registries; D058028:Research Report; D013030:Spain / Q000453:epidemiology; D015996:Survival Rate; D055815:Young Adult