Neuroendocrine tumors: fascination and infrequency

Abstract

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.

The diagnostic role of 68Ga-DOTATATE PET/CT in the detection of neuroendocrine tumours

“…Neuroendocrine tumours, particularly those of the gastro-intestinal tract, frequently express high levels of somatostatin receptors; therefore, radioisotope imaging techniques with somatostatin analogues labelled with 111 In or 99m Tc proved to be considerably more sensitive (60-90% depending on the tumour type) in visualization of lesions [3,[11][12][13][14].…”

From metastasis to the primary tumour: Giant liver metastasis from pancreatic neuroendocrine tumour

“…However, this can only be performed in 20 % of these patients (3). In our case, despite the large size of the liver metastasis and its increased aggressivity compared to the primary tumor, a full resection of the tumor was achieved, with a 5-year survival estimated higher than 60-80 % (4,5).…”

Diagnostic Performance of Endoscopic Ultrasound (EUS)/ Endoscopic Ultrasound – Fine Needle Aspiration (EUS-FNA) Cytology in Solid and Cystic Pancreatic Neuroendocrine Tumours

“…Earlier studies suggested that sensitivity might increase to 100% in cystic pNETs [20,24,25] if LBC and immunocytochemistry are employed. However, these were small studies (n=9) and more recent literature suggests a diagnostic yield varying between 60% and 70%.…”

“…24 No 1: 69-75 4 year and 2 month periods, to investigate the comparative performance between solid and cystic pNETs and to assess the value of EUS and EUS-FNA in individuals with a pancreatic mass not diagnosed as a pNET after cross-sectional imaging.…”