Neuroendocrine tumors: a population-based study of incidence and survival in Girona Province, 1994-2004.

BACKGROUND: Neuroendocrine tumors (NETs) belong to a rare and heterogeneous group of neoplasms with a wide range of aggressiveness. Little information is available about incidence and mortality of NETs in the World population. The objective of this study was to report the incidence and survival of these tumors in Girona Province. METHODS: We include all ICD-O3 codes that codified a NET, period 1994-2004. Data from malignant NETs came directly from the Girona Cancer Registry, and data from benign or with uncertain behavior NETs directly from the Anatomic-Pathological Laboratories. RESULTS: We identified 698 NETs, the most frequent were those aroused in the bronco-pulmonary system (65.75%), followed by the gastro-entero-pancreatics (GEP) (12.75%). Small cell lung carcinomas were the most frequent NETs of the bronco-pulmonary system (92.59%), with an age-standardized incidence rate (ASRw, world population standard) of 4.29/100,000 person-years. Carcinoid lung tumors had an ASRw of 0.32/100,000 person-years and a 5-year relative survival (RS) of 77%. The ASRw of GEP NETs was 1.07/100,000 person-years, and the 5-year RS was 95%. The 5-year RS of pancreas was 43%. Thyroid NETs had an ASRw of 0.17/100,000 person-years, and a 5-year RS of 75%. Pheocromocytomas had an ASRw of 0.47/100,000 person-years and a 5-year RS of 85%. Merkel cell carcinomas had an ASRw of 0.11/100,000 person-years, with a 5-year RS of 50%. CONCLUSION: To our knowledge, this is the first population-based study reporting incidence of NETs in Spain. Our data was consistent with other European reports. By providing the incidence and survival of NETs in Girona Province, this study contributes to a better understanding of these rare tumors.