1 / 21

Gastroenteropancreatic neuroendocrine tumors (GEP-NETs)

Gastroenteropancreatic neuroendocrine tumors (GEP-NETs). An update of epidemiological data. NET MASTERCLASS – 5 th July 2014 George Kanakis Consultant Endocrinologist Athens Naval & VA Hospital. Definition. heterogeneous group of tumors several NE cells of the GI tract

Download Presentation

Gastroenteropancreatic neuroendocrine tumors (GEP-NETs)

An Image/Link below is provided (as is) to download presentation Download Policy: Content on the Website is provided to you AS IS for your information and personal use and may not be sold / licensed / shared on other websites without getting consent from its author. Content is provided to you AS IS for your information and personal use only. Download presentation by click this link. While downloading, if for some reason you are not able to download a presentation, the publisher may have deleted the file from their server. During download, if you can't get a presentation, the file might be deleted by the publisher.

E N D

Presentation Transcript


  1. Gastroenteropancreatic neuroendocrine tumors (GEP-NETs) An update of epidemiological data NET MASTERCLASS – 5th July 2014 George Kanakis Consultant Endocrinologist Athens Naval & VA Hospital

  2. Definition • heterogeneous group of tumors • several NE cells of the GI tract • putative common precursor cell • ability to produce peptides / amines • cause distinct hormonal syndromes • variable biological behaviour • neuroendocrine cell of origin • anatomic site Vinik, Woltering, Neuroendocrine Tumors, 2006

  3. Nomenclature • Oberndorfer in 1907 first introduced the term “karzinoide” or cancer-like tumor • it does not convey the malignant potential of tumors • since then several names to describe the same tumor: apudoma / carcinoid / enterchromaffin cell tumor • lately the term “neuroendocrine neoplasm” has been widely accepted Modlin et al (2008) The Lancet

  4. Classification

  5. Data Sources • Autopsy Data for ALL NETs (Malmö– Sweden 1958-1969) • IR for ALL NETs about 8.4 per 100,000 • Surveillance Epidemiology and End Results (SEER) database • 7 262 696 cancer patients • 49,012 NETs - 25.531 GEP-NETs • National cancer registries of European Countries & Japan • Not Population Based National Registries: • Germany, France, Spain, Korea • Regional registries in Europe and in the USA • One prospective study in Austria in which: • all diagnoses of NETs in 1 yr (2004-2005) in the country recorded • the 2000 WHO classification was used • One systematic review: • IRs between 1958 and 2007 in different parts of the world • 33 papers included M Fraenkel et al. ERC (2014)21, R153–R163

  6. Incidence Rates M Fraenkel et al. ERC (2014)21, R153–R163

  7. Temporal Trends • GEP-NETs IR has increased steadily over the past four decades • 3.65-fold in the USA • 3.8 to 4.8-fold in the UK • The greatest increase • gastric and rectal NETs • The smallest increase • small intestine NETs • Annual Percent Change: 4.4% • exceeds that of any other malignancy Lawrenceet al. (2011) Endocr. & Metab. Clin. of N. America, 401–18

  8. Organ distribution

  9. Presentation Male : Female 1,08–1,5 : 1 Peak age at Dx: 50 – 70 y.o Gastric 60-64 Pancreas ~60 SINET 59-65 Colon 63-68 Rectal 52-57 Appendix 31-49 generally more indolent than carcinomas occasionally associated with a very aggressive clinical course Presentation symptoms related to tumor burden 46% Endocrine syndrome 23% Incidental diagnosis 29% Faggiano A et al (2012) J Endocrinol Invest.Oct;35(9):817-23

  10. Staging - Grading Niederle et al, Endocrine-Related Cancer(2010)17: 909–918

  11. Behavior vs origin ERC 2010; 17:909

  12. Survival 5 yr Survival rates: 45 – 82% SEER 2007: 64% Disease specific: 87% USA vs Europe

  13. Survival R A SI S C P Lawrenceet al. (2011) Endocr. & Metab. Clin. of N. America, 401–18

  14. Secondary Malignancies • SEER: “A higher incidence of further primary cancers compared to the general population (SIR: 1.15)” • German registry: “about 20% of GEP-NET patients developed one or more second malignancies” • More common in: • functional NETs • local / locoregional tumors Sherübl et al, World J Gastroenterol. 2013

  15. Specific Remarks I • Gastric Neuroendocrine Tumors • up to 23% of GEP-NETs • Type I tumors 75–80% • IRs now tend to be similar in males and females • Duodenal Neuroendocrine Tumors • 1.8- 3,8% of GEP-NETs • 40–60% have lymph node metastases • 15% associated with a functional syndrome • 10% ZES • 4% Carcinoid Syndrome

  16. Specific Remarks II • SI-NETs • the most common GEP-NETs in the Western world for years • low APC - recently surpassed by R-NETs in the USA • usually not approached with endoscopic techniques • male predominance • Appendiceal NETs • Goblet cell tumor was inconsistently included • often incidental finding @ appendicectomies (1,13%) • frequency is higher in females

  17. Specific Remarks III • Colon Tumours • predominance of right-sided NETs • 30–40% metastatic at the time of diagnosis • a low 5-year survival rate of about 43–50% • Rectal Tumours • IR increased 10X in 40 yrs • distant metastases occur in only 2.3% • an excellent five-year survival reaching about 90%

  18. Specific Remarks IV • Gastrinomas: • IR 0.5–2/ million population / year • Liver metastases much more frequent with pancreatic tumors • Insulinomas • IR 1–3/ million population / year • Less than 10% are malignant • Pancreatic Non-Functioning Tumors • 60–90% of PNETs • an increase in the incidental detection of NF-NETs • PFS is much greater for incidental NF-pNETs

  19. Key points • IR of GEP-NET has increased steadily over past 40 yrs • largest increases found in Gastric & Rectal NETs • Rectal NETs are the most common: • in east Asia • recently in the US SEER database • SI NET: still the most common in most European series • Appendiceal NETs: more common in young females • Pancreatic NETs: IR in registries dedicated to NETs • Five-year survival rates are: • highest in rectal and appendiceal NET • lowest in small intestinal and pancreatic NETs

  20. (Un)answered questions • Inconsistency in the nomenclature and classification of NETs • recent WHO classification criteria unify classification suggestions • Older cancer registries included only ‘malignant carcinoids’ • new ICD coding includes ‘benign’ NETs. This may explain some of the increase in the incidence of GEP-NETs • Registries document malignant diseases on the basis of clinical reports • recent prospective studies rely on strict histolological criteria • Data are still lacking in many parts of the world (Asia and Africa) • Population-based studies, ideally of whole countries • standardized collection of pathology reports • a uniform classification system, preferably the most updated WHO

  21. Thank you! geokan@endo.gr

More Related