In this article, I review and update of gastro-entero-pancreatic neuroendocrine tumors, which so much fascination have risen among healthcare providers on grounds of their infrequency, hormonal syndromes, and high survival rate, is performed based on references from the past fifteen years.
DEFINITIONNeuroendocrine tumors (NETs) or gastroenteropancreatic endocrine tumors (GEPETs) (2% of all gastrointestinal tumors) have their origin in tissues derived from the neural crest, neuroectoderm, and endoderm; 60-70% are carcinoid tumors, and 20-40% are found in the pancreas (NEPTs) ( Tables I and II). Gastrointestinal tract endocrine tumors other than carcinoid tumors may be gastrinomas, enteroglucagonomas, somatostatinomas, or non-functioning neuroendocrine tumors. Many of the data discussed herein have been taken from prior reviews (1-5).
INCIDENCEIn necropsies 0.5-1.5%, or even up to 5%, are NEPTs (1-2), many of them silent. They represent 1-3% of all pancreatic neoplasms.In Europe, in the one community-based study carried out thus far, incidence is smaller than one case per 100,000/year -specifically, 0.32 per 100,000/year for carcinoid tumors and 0.2 per 100,000/year (2 per million/year) in the pancreas (6). Insulinoma and gastrinoma, the most common types, have an incidence of 1 new case per million people and year. Glucagonoma and somatostatinoma are least common (1 per 20 and 40 million/year, respectively) (Table II).Carcinoids are twice as common in Afroamerican patients than among Caucasians (7).In the USA (8), incidence approaches 5 per million/year for NEPT, 1.8 in women and 2.6 in men, but is increasing and may well reach 10 per million people/year, with a higher frequency between 40 and 60 years of age, and in women.In our country the incidence of NEPT may be 0.08 per 100,000 inhabitants/year, or 1 new case every two years in Hospitalet de Llobregat (Barcelona).
GENETICSTumors may be sporadic, particularly carcinoids, or may develop in association with dominant autosomic syndromes (2) -locus 11q13 for MEN-1 -including multiple REV ESP ENFERM DIG (Madrid) Vol. 101. N.°3, pp. 195-208, 2009 Received: 28-12-08. Accepted: 08-01-09.