Epidemiology and survival in patients with carcinoid disease in the Netherlands - An epidemiological study with 2391 patients

Background: Carcinoid tumours are rare malignant neuro-endocrine tumours. In 1992 octreotide was introduced in the Netherlands as a palliative treatment for the carcinoid syndrome in metastatic carcinoid disease. The aims of this epidemiological study were to evaluate epidemiological data and the impact of octreotide on survival in metastatic carcinoid disease. Patients and methods: Between 1989 and 1996, 2391 patients with carcinoid disease were diagnosed in the Netherlands. Survival data from two Registries were available in 619 patients, diagnosed between 1980 and 1997. Results: Between 1989-1996, incidence was 1.95/100,000. Under the age of 50 years a significant female predominance was observed. Under the age of 35 years, appendiceal carcinoid was the most frequently diagnosed primary site. Incidence of distant metastases at diagnosis for appendix and lung primary sites was 1.6% and 5.5%, compared to 40% in the other primary sites. Multivariate analysis of 619 patients revealed that age, stage and appendix localisation predicted survival. In metastatic disease, however, only year of diagnosis after 1992 independently predicted survival (P = 0.012). Conclusions: The female predominance found under the age of 50 years suggests hormonal influence. Improved survival in metastatic carcinoid disease might relate to the use of octreotide.