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Reviews in Endocrine and Metabolic Disorders

Published in:

01-12-2017

Hormone secreting gastro-entero-pancreatic neuroendocrine neoplasias (GEP-NEN): When to consider, how to diagnose?

Authors: Christian Fottner, Martina Ferrata, Matthias M. Weber

Published in: Reviews in Endocrine and Metabolic Disorders | Issue 4/2017

Abstract

Neuroendocrine neoplasms of the digestive system (GEP-NEN) represent a heterogeneous group of malignancies with various clinical presentation and prognosis. GEP-NENs can potentially affect all organs of the gastrointestinal tract; characteristically they share the biological property to produce and secrete peptides and neuroamines. About 30% of GEP-NENs are hormonally active and can cause specific clinical syndromes. The clinical presentation mainly depends on the primary site of the tumor and its functionality. Because of the wide spectrum of clinical symptoms and their misperceived rarity, diagnosis of GEP-NENs is often delayed for years and tumors are detected first in an advanced stage. Early identification of a specific hormonal syndrome can significantly impact tumor diagnosis and treatment, moreover the preoperative management of NEN hormonal release avoids potential life threatening hormonal crisis. However, GEP-NEN diagnostic work-up is challenging, it requires a multidisciplinary team and needs particular experience; standardized protocols and clinical experience are essential for a proper endocrine diagnostic work-up. In addition to the biochemical diagnostic, further radiologic and endoscopic imaging modalities are required moreover, somatostatin-receptor based functional imaging, using either Octreotide-scintigraphy or novel PET-based techniques with specific isotopes like Ga⁶⁸-DOTA-octreotate, plays an important role for the detection of the primary tumor as well as for the evaluation of the tumor extent.

Ahlman H, Nilsson. The gut as the largest endocrine organ in the body. Ann Oncol. 2001;12(Suppl 2):S63–8. https://doi.org/10.1093/annonc/12.suppl_2.S63.CrossRefPubMed

Yao JC, Hassan M, Phan A, Dagohoy C, Leary C, Mares JE, 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(18):3063–72. https://doi.org/10.1200/JCO.2007.15.4377.CrossRefPubMed

Hemminki K, Li X. Familial carcinoid tumors and subsequent cancers: a nation-wide epidemiologic study from Sweden. Int J Cancer. 2001;94(3):444–8. https://doi.org/10.1002/ijc.1473.CrossRefPubMed

Levi F, Te VC, Randimbison L, Rindi G, La Vecchia C. Epidemiology of carcinoid neoplasms in Vaud, Switzerland, 1974-97. Br J Cancer. 2000;83(7):952–5. https://doi.org/10.1054/bjoc.2000.1394.CrossRefPubMedPubMedCentral

Modlin IM, Oberg K, Chung DC, Jensen RT, de Herder WW, Thakker RV, et al. Gastroenteropancreatic neuroendocrine tumours. Lancet Oncol. 2008;9(1):61–72. https://doi.org/10.1016/S1470-2045(07)70410-2.CrossRefPubMed

Modlin IM, Lye KD, Kidd M. A 5-decade analysis of 13,715 carcinoid tumors. Cancer. 2003;97(4):934–59. https://doi.org/10.1002/cncr.11105.CrossRefPubMed

Rindi G, Wiedenmann B. Neuroendocrine neoplasms of the gut and pancreas: new insights. Nat Rev Endocrinol. 2011;8(1):54–64. https://doi.org/10.1038/nrendo.2011.120.CrossRefPubMed

Lam JC, Xu A, Tam S, et al. Hypoadiponectinemia is related to sympathetic activation and severity of obstructive sleep apnea. Sleep. 2008;31(12):1721–7. https://doi.org/10.1093/sleep/31.12.1721.CrossRefPubMedPubMedCentral

Bosman FTCF, Hruban RH, Theise ND. World Health Organization (WHO) classification of Tumours of the digestive system. 4th ed. Geneva: WHO Press; 2010.

10.

Tang LH, Basturk O, Sue JJ, Klimstra DSA. Practical approach to the classification of WHO grade 3 (G3) well-differentiated neuroendocrine tumor (WD-NET) and poorly differentiated neuroendocrine carcinoma (PD-NEC) of the pancreas. Am J Surg Pathol. 2016;40(9):1192–202. https://doi.org/10.1097/PAS.0000000000000662.CrossRefPubMedPubMedCentral

11.

Karges W, Adler G. Clinical genetics of neuroendocrine tumors. Med Klin (Munich). 2003;98(12):712–6. https://doi.org/10.1007/s00063-003-1317-2.CrossRef

12.

Anlauf M, Garbrecht N, Bauersfeld J, et al. Hereditary neuroendocrine tumors of the gastroenteropancreatic system. Virchows Arch. 2007;451(Suppl 1):S29–38.CrossRefPubMed

13.

Thakker RV, Newey PJ, Walls GV, Bilezikian J, Dralle H, Ebeling PR, et al. Clinical practice guidelines for multiple endocrine neoplasia type 1 (MEN1). J Clin Endocrinol Metab. 2012;97(9):2990–3011. https://doi.org/10.1210/jc.2012-1230.CrossRefPubMed

14.

Halfdanarson TR, Rubin J, Farnell MB, Grant CS, Petersen GM. Pancreatic endocrine neoplasms: epidemiology and prognosis of pancreatic endocrine tumors. Endocr Relat Cancer. 2008;15(2):409–27. https://doi.org/10.1677/ERC-07-0221.CrossRefPubMedPubMedCentral

15.

Klimstra DS, Modlin IR, Adsay NV, Chetty R, Deshpande V, Gönen M, et al. Pathology reporting of neuroendocrine tumors: application of the Delphic consensus process to the development of a minimum pathology data set. Am J Surg Pathol. 2010;34(3):300–13. https://doi.org/10.1097/PAS.0b013e3181ce1447.CrossRefPubMed

16.

Hofer MD, Chang MC, Hirko KA, Rubin MA, Nose V. Immunohistochemical and clinicopathological correlation of the metastasis-associated gene 1 (MTA1) expression in benign and malignant pancreatic endocrine tumors. Mod Pathol. 2009;22(7):933–9. https://doi.org/10.1038/modpathol.2009.50.CrossRefPubMed

17.

Modlin IM, Moss SF, Gustafsson BI, Lawrence B, Schimmack S, Kidd M. The archaic distinction between functioning and nonfunctioning neuroendocrine neoplasms is no longer clinically relevant. Langenbeck's Arch Surg. 2011;396(8):1145–56. https://doi.org/10.1007/s00423-011-0794-7.CrossRef

18.

Oberg K, Eriksson B. Endocrine tumours of the pancreas. Best Pract Res Clin Gastroenterol. 2005;19(5):753–81. https://doi.org/10.1016/j.bpg.2005.06.002.CrossRefPubMed

19.

Ramage JK, Ahmed A, Ardill J, Bax N, Breen DJ, Caplin ME, et al. Guidelines for the management of gastroenteropancreatic neuroendocrine (including carcinoid) tumours (NETs). Gut. 2012;61(1):6–32. https://doi.org/10.1136/gutjnl-2011-300831.CrossRefPubMed

20.

Niederle B, Pape UF, Costa F, Gross D, Kelestimur F, Knigge U, et al. ENETS consensus guidelines update for neuroendocrine neoplasms of the jejunum and ileum. Neuroendocrinology. 2016;103(2):125–38. https://doi.org/10.1159/000443170.CrossRefPubMed

21.

Srirajaskanthan R, Ahmed A, Prachialias A, Srinivasan P, Heaton N, Jervis N, et al. ENETS TNM staging predicts prognosis in small bowel neuroendocrine Tumours. ISRN Oncol. 2013;2013:420795. https://doi.org/10.1155/2013/420795.PubMedPubMedCentral

22.

Tellez MR, Mamikunian G, O'Dorisio TM, Vinik AI, Woltering EA. Single fasting plasma 5-HIAA value correlates with 24-hour urinary 5-HIAA values and other biomarkers in midgut neuroendocrine tumors (NETs). Pancreas. 2013;42(3):405–10. https://doi.org/10.1097/MPA.0b013e318271c0d5.CrossRefPubMed

23.

Adaway JE, Dobson R, Walsh J, Cuthbertson DJ, Monaghan PJ, Trainer PJ, et al. Serum and plasma 5-hydroxyindoleacetic acid as an alternative to 24-h urine 5-hydroxyindoleacetic acid measurement. Ann Clin Biochem. 2016;53(Pt 5):554–60. https://doi.org/10.1177/0004563215613109.CrossRefPubMed

24.

Gregg Mamikunian AIV, O’Dorisio TM, Woltering EA, Go VLW. Neuroendocrine tumora a comprehensive guide to diagnosis and management fourth edition. Inter Science Institute; 2009.

25.

Hannah-Shmouni F, Stratakis CA, Koch CA. Flushing in (neuro)endocrinology. Rev Endocr Metab Disord. 2016;17(3):373–80. https://doi.org/10.1007/s11154-016-9394-8.CrossRefPubMedPubMedCentral

26.

Okabayashi T, Shima Y, Sumiyoshi T, Kozuki A, Ito S, Ogawa Y, et al. Diagnosis and management of insulinoma. World J Gastroenterol. 2013;19(6):829–37. https://doi.org/10.3748/wjg.v19.i6.829.CrossRefPubMedPubMedCentral

27.

Marks V. Recognition and differential diagnosis of spontaneous hypoglycaemia. Clin Endocrinol. 1992;37(4):309–16. https://doi.org/10.1111/j.1365-2265.1992.tb02329.x.CrossRef

28.

Falconi M, Eriksson B, Kaltsas G, Bartsch DK, Capdevila J, Caplin M, et al. ENETS consensus guidelines update for the Management of Patients with functional pancreatic neuroendocrine tumors and non-functional pancreatic neuroendocrine tumors. Neuroendocrinology. 2016;103(2):153–71. https://doi.org/10.1159/000443171.CrossRefPubMedPubMedCentral

29.

Cryer PE, Axelrod L, Grossman AB, Heller SR, Montori VM, Seaquist ER, et al. Evaluation and management of adult hypoglycemic disorders: an Endocrine Society clinical practice guideline. J Clin Endocrinol Metab. 2009;94(3):709–28. https://doi.org/10.1210/jc.2008-1410.CrossRefPubMed

30.

Vezzosi D, Bennet A, Fauvel J, Caron P. Insulin, C-Peptide and proinsulin for the biochemical diagnosis of hypoglycaemia related to endogenous hyperinsulinism. Eur J Endocrinol. 2007;157(1):75–83. https://doi.org/10.1530/EJE-07-0109.CrossRefPubMed

31.

Service FJ, McMahon MM, O'Brien PC, Ballard DJ. Functioning insulinoma--incidence, recurrence, and long-term survival of patients: a 60-year study. Mayo Clin Proc. 1991;66(7):711–9. https://doi.org/10.1016/S0025-6196(12)62083-7.CrossRefPubMed

32.

Anlauf M, Bauersfeld J, Raffel A, Koch CA, Henopp T, Alkatout I, et al. Insulinomatosis: a multicentric insulinoma disease that frequently causes early recurrent hyperinsulinemic hypoglycemia. Am J Surg Pathol. 2009;33(3):339–46. https://doi.org/10.1097/PAS.0b013e3181874eca.CrossRefPubMed

33.

Dizon AM, Kowalyk S, Hoogwerf BJ. Neuroglycopenic and other symptoms in patients with insulinomas. Am J Med. 1999;106(3):307–10. https://doi.org/10.1016/S0002-9343(99)00021-2.CrossRefPubMed

34.

Guettier JM, Lungu A, Goodling A, Cochran C, Gorden P. The role of proinsulin and insulin in the diagnosis of insulinoma: a critical evaluation of the Endocrine Society clinical practice guideline. J Clin Endocrinol Metab. 2013;98(12):4752–8. https://doi.org/10.1210/jc.2013-2182.CrossRefPubMedPubMedCentral

35.

Service FJ, Natt N. The prolonged fast. J Clin Endocrinol Metab. 2000;85(11):3973–4. https://doi.org/10.1210/jcem.85.11.6934.CrossRefPubMed

36.

Vezzosi D, Walter T, Laplanche A, Raoul JL, Dromain C, Ruszniewski P, et al. Chromogranin A measurement in metastatic well-differentiated gastroenteropancreatic neuroendocrine carcinoma: screening for false positives and a prospective follow-up study. Int J Biol Markers. 2011;26(2):94–101. https://doi.org/10.5301/JBM.2011.8327.CrossRefPubMed

37.

Hirshberg B, Skarulis MC, Pucino F, Csako G, Brennan R, Gorden P. Repaglinide-induced factitious hypoglycemia. J Clin Endocrinol Metab. 2001;86(2):475–7. https://doi.org/10.1210/jcem.86.2.7160.CrossRefPubMed

38.

Freckmann G, Schmid C, Baumstark A, Pleus S, Link M, Haug C. System accuracy evaluation of 43 blood glucose monitoring systems for self-monitoring of blood glucose according to DIN EN ISO 15197. J Diabetes Sci Technol. 2012;6(5):1060–75. https://doi.org/10.1177/193229681200600510.CrossRefPubMedPubMedCentral

39.

Eisenberg D, Azagury DE, Ghiassi S, Grover BT, Kim JJASMBS. Position statement on postprandial Hyperinsulinemic hypoglycemia after bariatric surgery. Surg Obes Relat Dis. 2017;13(3):371–8. https://doi.org/10.1016/j.soard.2016.12.005.CrossRefPubMed

40.

Roy PK, Venzon DJ, Shojamanesh H, Abou-Saif A, Peghini P, Doppman JL, et al. Zollinger-Ellison syndrome. Clinical presentation in 261 patients. Medicine (Baltimore). 2000;79(6):379–411. https://doi.org/10.1097/00005792-200011000-00004.CrossRef

41.

Rindi G, Kloppel G, Alhman H, et al. TNM staging of foregut (neuro)endocrine tumors: a consensus proposal including a grading system. Virchows Arch. 2006;449(4):395–401. https://doi.org/10.1007/s00428-006-0250-1.CrossRefPubMedPubMedCentral

42.

Jensen RT, Cadiot G, Brandi ML, de Herder WW, Kaltsas G, Komminoth P, et al. ENETS consensus guidelines for the management of patients with digestive neuroendocrine neoplasms: functional pancreatic endocrine tumor syndromes. Neuroendocrinology. 2012;95(2):98–119. https://doi.org/10.1159/000335591.CrossRefPubMedPubMedCentral

43.

Wilson SD, Doffek KM, Krzywda EA, Quebbeman EJ, Christians KK, Pappas SG. Zollinger-Ellison syndrome associated with a history of alcohol abuse: coincidence or consequence? Surgery. 2011;150(6):1129–35. https://doi.org/10.1016/j.surg.2011.09.004.CrossRefPubMed

44.

Poitras P, Gingras MH, Rehfeld JF. The Zollinger-Ellison syndrome: dangers and consequences of interrupting antisecretory treatment. Clin Gastroenterol Hepatol. 2012;10(2):199–202. https://doi.org/10.1016/j.cgh.2011.08.012.CrossRefPubMed

45.

Berna MJ, Hoffmann KM, Long SH, Serrano J, Gibril F, Jensen RT. Serum gastrin in Zollinger-Ellison syndrome: II. Prospective study of gastrin provocative testing in 293 patients from the National Institutes of Health and comparison with 537 cases from the literature. Evaluation of diagnostic criteria, proposal of new criteria, and correlations with clinical and tumoral features. Medicine (Baltimore). 2006;85(6):331–64. https://doi.org/10.1097/MD.0b013e31802b518c.CrossRef

46.

McGuigan JE, Wolfe MM. Secretin injection test in the diagnosis of gastrinoma. Gastroenterology. 1980;79(6):1324–31.PubMed

47.

O'Toole D, Grossman A, Gross D, et al. ENETS consensus guidelines for the standards of Care in Neuroendocrine Tumors: biochemical markers. Neuroendocrinology. 2009;90(2):194–202. https://doi.org/10.1159/000225948.CrossRefPubMed

48.

Hubbard H, Goodgame R. Evaluation of possible gastrinoma. MedGenMed. 2007;9(1):31.PubMedPubMedCentral

49.

Berna MJ, Hoffmann KM, Serrano J, Gibril F, Jensen RT. Serum gastrin in Zollinger-Ellison syndrome: I. Prospective study of fasting serum gastrin in 309 patients from the National Institutes of Health and comparison with 2229 cases from the literature. Medicine (Baltimore). 2006;85(6):295–330. https://doi.org/10.1097/01.md.0000236956.74128.76.CrossRef

50.

Ito T, Cadiot G, Jensen RT. Diagnosis of Zollinger-Ellison syndrome: increasingly difficult. World J Gastroenterol. 2012;18(39):5495–503. https://doi.org/10.3748/wjg.v18.i39.5495.CrossRefPubMedPubMedCentral

51.

Sanchez W. Mayo clinic gastroenterology and hepatology board review. Oxford University Press, Incorporate; 2014 28 ago, p. 57–72.

52.

Mansour JC, Chen H. Pancreatic endocrine tumors. J Surg Res. 2004;120(1):139–61. https://doi.org/10.1016/j.jss.2003.12.007.CrossRefPubMed

53.

Singer J, Werner F, Koch CA, et al. Ectopic Cushing's syndrome caused by a well differentiated ACTH-secreting neuroendocrine carcinoma of the ileum. Exp Clin Endocrinol Diabetes. 2010;118(8):524–9. https://doi.org/10.1055/s-0029-1243634.CrossRefPubMed

54.

Pape UF, Maasberg S, Jann H, Pschowski R, Krüger S, Prasad V, et al. Management of follow-up of neuroendocrine neoplasias. Best Pract Res Clin Endocrinol Metab. 2016;30(1):129–40. https://doi.org/10.1016/j.beem.2016.02.001.CrossRefPubMed

55.

Pacak K, Jochmanova I, Prodanov T, Yang C, Merino MJ, Fojo T, et al. New syndrome of paraganglioma and somatostatinoma associated with polycythemia. J Clin Oncol. 2013;31(13):1690–8. https://doi.org/10.1200/JCO.2012.47.1912.CrossRefPubMedPubMedCentral

56.

Campana D, Nori F, Piscitelli L, Morselli-Labate AM, Pezzilli R, Corinaldesi R, et al. Chromogranin A: is it a useful marker of neuroendocrine tumors? J Clin Oncol. 2007;25(15):1967–73. https://doi.org/10.1200/JCO.2006.10.1535.CrossRefPubMed

57.

Chou WC, Hung YS, Hsu JT, Chen JS, Lu CH, Hwang TL, et al. Chromogranin A is a reliable biomarker for gastroenteropancreatic neuroendocrine tumors in an Asian population of patients. Neuroendocrinology. 2012;95(4):344–50. https://doi.org/10.1159/000333853.CrossRefPubMed

58.

Nehar D, Lombard-Bohas C, Olivieri S, Claustrat B, Chayvialle JA, Penes MC, et al. Interest of chromogranin A for diagnosis and follow-up of endocrine tumours. Clin Endocrinol. 2004;60(5):644–52. https://doi.org/10.1111/j.1365-2265.2004.02030.x.CrossRef

59.

Tomassetti P, Migliori M, Simoni P, Casadei R, de Iasio R, Corinaldesi R, et al. Diagnostic value of plasma chromogranin A in neuroendocrine tumours. Eur J Gastroenterol Hepatol. 2001;13(1):55–8. https://doi.org/10.1097/00042737-200101000-00010.CrossRefPubMed

60.

Gustafsson BI, Kidd M, Modlin IM. Neuroendocrine tumors of the diffuse neuroendocrine system. Curr Opin Oncol. 2008;20(1):1–12. https://doi.org/10.1097/CCO.0b013e3282f1c595.CrossRefPubMed

61.

Modlin IM, Gustafsson BI, Moss SF, Pavel M, Tsolakis AV, Kidd M. Chromogranin A--biological function and clinical utility in neuro endocrine tumor disease. Ann Surg Oncol. 2010;17(9):2427–43. https://doi.org/10.1245/s10434-010-1006-3.CrossRefPubMed

62.

Lawrence B, Gustafsson BI, Kidd M, Pavel M, Svejda B, Modlin IM. The clinical relevance of chromogranin A as a biomarker for gastroenteropancreatic neuroendocrine tumors. Endocrinol Metab Clin N Am. 2011;40(1):111–134, viii. https://doi.org/10.1016/j.ecl.2010.12.001.CrossRef

63.

Stridsberg M, Eriksson B, Oberg K, Janson ETA. Comparison between three commercial kits for chromogranin A measurements. J Endocrinol. 2003;177(2):337–41. https://doi.org/10.1677/joe.0.1770337.CrossRefPubMed

64.

Modlin IM, Kidd M, Latich I, Zikusoka MN, Shapiro MD. Current status of gastrointestinal carcinoids. Gastroenterology. 2005;128(6):1717–51. https://doi.org/10.1053/j.gastro.2005.03.038.CrossRefPubMed

65.

Qiao XW, Qiu L, Chen YJ, Meng CT, Sun Z, Bai CM, et al. Chromogranin A is a reliable serum diagnostic biomarker for pancreatic neuroendocrine tumors but not for insulinomas. BMC Endocr Disord. 2014;14(1):64. https://doi.org/10.1186/1472-6823-14-64.CrossRefPubMedPubMedCentral

66.

Gut P, Czarnywojtek A, Fischbach J, Bączyk M, Ziemnicka K, Wrotkowska E, et al. Chromogranin A - unspecific neuroendocrine marker. Clinical utility and potential diagnostic pitfalls. Arch Med Sci. 2016;12(1):1–9. https://doi.org/10.5114/aoms.2016.57577.CrossRefPubMedPubMedCentral

67.

Ardill JE, O'Dorisio TM. Circulating biomarkers in neuroendocrine tumors of the enteropancreatic tract: application to diagnosis, monitoring disease, and as prognostic indicators. Endocrinol Metab Clin N Am. 2010;39(4):777–90. https://doi.org/10.1016/j.ecl.2010.09.001.CrossRef

68.

Knigge U, Capdevila J, Bartsch DK, Baudin E, Falkerby J, Kianmanesh R, Kos-Kudla B, Niederle B, Nieveen van Dijkum E, O'Toole D, Pascher A, Reed N, Sundin A, Vullierme MP. Antibes Consensus Conference Participants; Antibes Consensus Conference participants. ENETS Consensus Recommendations for the Standards of Care in Neuroendocrine Neoplasms: Follow-Up and Documentation. Neuroendocrinology. 2017;105(3):310–19. https://doi.org/10.1159/000458155.

69.

Panzuto F, Severi C, Cannizzaro R, Falconi M, Angeletti S, Pasquali A, et al. Utility of combined use of plasma levels of chromogranin A and pancreatic polypeptide in the diagnosis of gastrointestinal and pancreatic endocrine tumors. J Endocrinol Investig. 2004;27(1):6–11. https://doi.org/10.1007/BF03350903.CrossRef

70.

Walter T, Chardon L, Chopin-laly X, Raverot V, Caffin AG, Chayvialle JA, et al. Is the combination of chromogranin A and pancreatic polypeptide serum determinations of interest in the diagnosis and follow-up of gastro-entero-pancreatic neuroendocrine tumours? Eur J Cancer. 2012;48(12):1766–73. https://doi.org/10.1016/j.ejca.2011.11.005.CrossRefPubMed

71.

Anderson MA, Carpenter S, Thompson NW, Nostrant TT, Elta GH, Scheiman JM. Endoscopic ultrasound is highly accurate and directs management in patients with neuroendocrine tumors of the pancreas. Am J Gastroenterol. 2000;95(9):2271–7. https://doi.org/10.1111/j.1572-0241.2000.02480.x.CrossRefPubMed

72.

Hoeffel C, Job L, Ladam-Marcus V, Vitry F, Cadiot G, Marcus C. Detection of hepatic metastases from carcinoid tumor: prospective evaluation of contrast-enhanced ultrasonography. Dig Dis Sci. 2009;54(9):2040–6. https://doi.org/10.1007/s10620-008-0570-x.CrossRefPubMed

73.

Ramage JK, Davies AH, Ardill J, et al. Guidelines for the management of gastroenteropancreatic neuroendocrine (including carcinoid) tumours. Gut. 2005;54(Suppl 4):iv1–16.PubMedPubMedCentral

74.

Gouya H, Vignaux O, Augui J, Dousset B, Palazzo L, Louvel A, et al. CT, endoscopic sonography, and a combined protocol for preoperative evaluation of pancreatic insulinomas. AJR Am J Roentgenol. 2003;181(4):987–92. https://doi.org/10.2214/ajr.181.4.1810987.CrossRefPubMed

75.

Bailey AA, Debinski HS, Appleyard MN, Remedios ML, Hooper JE, Walsh AJ, et al. Diagnosis and outcome of small bowel tumors found by capsule endoscopy: a three-center Australian experience. Am J Gastroenterol. 2006;101(10):2237–43. https://doi.org/10.1111/j.1572-0241.2006.00749.x.CrossRefPubMed

76.

Bellutti M, Fry LC, Schmitt J, Seemann M, Klose S, Malfertheiner P, et al. Detection of neuroendocrine tumors of the small bowel by double balloon enteroscopy. Dig Dis Sci. 2009;54(5):1050–8. https://doi.org/10.1007/s10620-008-0456-y.CrossRefPubMed

77.

Miederer M, Weber MM, Fottner C. Molecular imaging of gastroenteropancreatic neuroendocrine tumors. Gastroenterol Clin N Am. 2010;39(4):923–35. https://doi.org/10.1016/j.gtc.2010.08.031.CrossRef

78.

Guettier JM, Kam A, Chang R, Skarulis MC, Cochran C, Alexander HR, et al. Localization of insulinomas to regions of the pancreas by intraarterial calcium stimulation: the NIH experience. J Clin Endocrinol Metab. 2009;94(4):1074–80. https://doi.org/10.1210/jc.2008-1986.CrossRefPubMedPubMedCentral

Title: Hormone secreting gastro-entero-pancreatic neuroendocrine neoplasias (GEP-NEN): When to consider, how to diagnose?
Authors: Christian Fottner
Martina Ferrata
Matthias M. Weber
Publication date: 01-12-2017
Publisher: Springer US
Published in: Reviews in Endocrine and Metabolic Disorders / Issue 4/2017
Print ISSN: 1389-9155
Electronic ISSN: 1573-2606
DOI: https://doi.org/10.1007/s11154-017-9438-8

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