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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 4,1/100.000. Rammer begge kjønn. Gjennomsnittsalder ved diagnose er 62 år
Symptomer:
Er relatert til svulstens lokalisasjon og/eller hormonproduksjon
Funn:
Kliniske 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. Federspiel BH, Hansen CP, Hasselby JP, Bardram L, Knigge U. Incidens, patologi og klinik ved neuroendokrine gastrointestinale tumorer. Ugeskr Læger 2010; 172: 2946. Ugeskrift for Læger  
  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. Rindi G, Klöppel G, Couvelard A, et al. TNM staging of midgut and hindgut (neuro) endocrine tumors: a consensus proposal including a grading system. Virchows Arch 2007; 451: 757-62. PubMed  
  5. L H Sobin; M K Gospodarowicz; Ch Wittekind; International Union against Cancer. TNM classification of malignant tumours. Chichester, West Sussex, UK ; Hoboken, NJ: Wiley-Blackwell, 2007.
  6. Hauso O, Gustafsson BI, Kidd M, Waldum HL, et. al. Neuroendocrine tumor epidemiology: contrasting Norway and North America. Cancer 2008; 113: 2655-64. PubMed  
  7. Yao JC, Hassan M, Phan A, et al. One hundred years after "carcinoid": epidemiology of and prognostic factors for neuroendocrine tumors in 35,825 cases in the United States. J Clin Oncol 2008; 26: 3063-72. PubMed  
  8. 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  
  9. Habal N, Sims C, Bilchik AJ. Gastrointestinal carcinoid tumors and second primary malignancies. J Surg Oncol 2000; 75: 310-6. PubMed  
  10. Tichansky DS, Cagir B, Borrazzo E, et al. Risk of second cancers in patients with colorectal carcinoids. Dis Colon Rectum 2002; 45: 91-7. PubMed  
  11. 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  
  12. 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  
  13. 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  
  14. 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  
  15. Hansen CP, Knigge U.. Biotherapy of neuroendocrine tumours of the gastrointestinal tract and pancreas. Ugeskr Laeger 2008; 170: 2145-8. PubMed  
  16. 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  
  17. Baez JC, Jagannathan JP, Krajewski K, et al. Pheochromocytoma and paraganglioma: imaging characteristics. Cancer Imaging 2012; 7: 153-62. PubMed  
  18. 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  
  19. 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  
  20. 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  
  21. 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  
  22. European Neuroendocrine Tumors Society. Nettside besøkt 07.07.12 www.enets.org  
  23. 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