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Nevroendokrine svulster (NET)

Sist revidert:
Sist revidert av:
Definisjon:
Nevroendokrine svulster har uklar etiologi, opptrer vanligvis sporadisk, men de kan være familiære
Forekomst:
I Norge er prevalens på 4/100.000. Rammer begge kjønn. Gjennomsnittsalder ved diagnose er 62 år
Symptomer:
Er relatert til svulstens lokalisasjon og/eller hormonproduksjon
Funn:
Svulstene er ofte store ved diagnosetidspunktet. Tarmobstruksjon eller palpable tumormasser er derfor mulig
Diagnostikk:
Blodprøver av tumormarkører og bildeundersøkelser i utredningen, histologi verifiserer
Behandling:
Kun kirurgi kan kurere tilstanden, men andre typer behandling har livsforlengende og palliativ effekt
  1. Helsedirektoratet. Nevroendokrine svulster i fordøyelsessystemet – handlingsprogram. Sist faglig oppdatert: 20. oktober 2020. www.helsedirektoratet.no  
  2. Thiis-Evensen E. Nevroendokrine pancreassvulster. Gastroenterologen, 23.05.2012. gastroenterologen.no  
  3. Rindi G, Klimstra DS, Abedi-Ardekani B, et al. A common classification framework for neuroendocrine neoplasms: an International Agency for Research on Cancer (IARC) and World Health Organization (WHO) expert consensus proposal. Mod Pathol 2018; 31:1770. PubMed  
  4. Amin MB, Greene FL, Edge SB, et al. The Eighth Edition AJCC Cancer Staging Manual: Continuing to build a bridge from a population-based to a more "personalized" approach to cancer staging. CA Cancer J Clin. 2017;67(2):93-99. PubMed  
  5. Hauso O, Gustafsson BI, Kidd M, Waldum HL, et. al. Neuroendocrine tumor epidemiology: contrasting Norway and North America. Cancer 2008; 113: 2655-64. PubMed  
  6. Ito T, Sasano H, Tanaka M, Osamura RY,et. al. Epidemiological study of gastroenteropancreatic neuroendocrine tumors in Japan. J Gastroenterol 2010; 45: 234-43. PubMed  
  7. Pirozzi A, Riccardi F, Arpino G, et al. Occurrence of second primary malignancies in patients with neuroendocrine tumors of the digestive tract: A case report. Medicine (Baltimore). 2019 Jul;98(29):e16508. PMID: 31335724 PubMed  
  8. Delle Fave G, Kwekkeboom DJ, Van Cutsem E, et al. ENETS Consensus Guidelines for the management of patients with gastroduodenal neoplasms. Neuroendocrinology 2012; 95: 74-87. PubMed  
  9. Pape UF, Perren A, Niederle B, et al. ENETS Consensus Guidelines for the management of patients with neuroendocrine neoplasms from the jejuno-ileum and the appendix including goblet cell carcinomas. Neuroendocrinology 2010; 95: 135-56. PubMed  
  10. Caplin M, Sundin A, Nillson O, et al. ENETS Consensus Guidelines for the management of patients with digestive neuroendocrine neoplasms: colorectal neuroendocrine neoplasms. Neuroendocrinology 2012; 95: 88-97. PubMed  
  11. Modlin IM, Gustafsson BI, Moss SF, et al. Chromogranin A--biological function and clinical utility in neuro endocrine tumor disease. Ann Surg Oncol 2010; 17: 2427-43. PubMed  
  12. Falconi M, Bartsch DK, Eriksson B, et al. ENETS Consensus Guidelines for the management of patients with digestive neuroendocrine neoplasms of the digestive system: well-differentiated pancreatic non-functioning tumors. Neuroendocrinology 2010; 95: 120-34. PubMed  
  13. Baez JC, Jagannathan JP, Krajewski K, et al. Pheochromocytoma and paraganglioma: imaging characteristics. Cancer Imaging 2012; 7: 153-62. PubMed  
  14. Pavel M, Baudin E, Couvelard A, et al. ENETS Consensus Guidelines for the management of patients with liver and other distant metastases from neuroendocrine neoplasms of foregut, midgut, hindgut, and unknown primary. Neuroendocrinology 2012; 95: 157-76. PubMed  
  15. Strosberg J, El-Haddad G, Wolin E et al. Phase 3 Trial of 177Lu-Dotatate for Midgut Neuroendocrine Tumors. N Engl J Med 2017; 376(2): 125-35. pmid:28076709 PubMed  
  16. Jensen RT, Cadiot G, Brandi ML, et al. ENETS Consensus Guidelines for the management of patients with digestive neuroendocrine neoplasms: functional pancreatic endocrine tumor syndromes. Neuroendocrinology 2012; 95: 98-119. PubMed  
  17. Janson ET, Sørbye H, Welin S, et al. Nordic Guidelines 2010 for diagnosis and treatment of gastroenteropancreatic neuroendocrine tumours. Acta Oncol 2010; 49: 740-56. PubMed  
  18. European Neuroendocrine Tumors Society. Nettside besøkt 07.07.12 www.enets.org  
  19. Goede AC, Caplin ME, Winslet MC. Carcinoid tumour of the appendix. Br J Surg 2003; 90: 1317-22. PubMed  
  • Terje Johannessen, professor i allmennmedisin, Trondheim

Tidligere fagmedarbeidere

  • Sven-Petter Haugvik, gastrokirurg, lege og forsker, Gastrokirurgisk avdeling, Oslo universitetssykehus, Rikshospitalet
  • Lars Aabakken, førsteamanuensis ved Universitetet i Oslo, lege ved Medisinsk avdeling, Rikshospitalet