Advances in Clinical and Experimental Medicine

Title abbreviation: Adv Clin Exp Med
5-Year IF – 2.0, IF – 1.9, JCI (2024) – 0.43
Scopus CiteScore – 4.3
Q1 in SJR 2024, SJR score – 0.598, H-index: 49 (SJR)
ICV – 161.00; MNiSW – 70 pts
Initial editorial assessment and first decision within 24 h

ISSN 1899–5276 (print), ISSN 2451-2680 (online)
Periodicity – monthly

Download original text (EN)

Advances in Clinical and Experimental Medicine

2020, vol. 29, nr 2, February, p. 265–270

doi: 10.17219/acem/111806

Publication type: review

Language: English

License: Creative Commons Attribution 3.0 Unported (CC BY 3.0)

Download citation:

  • BIBTEX (JabRef, Mendeley)
  • RIS (Papers, Reference Manager, RefWorks, Zotero)

Neuroendocrine tumors of the gastrointestinal tract and pancreas: Is it also a challenge for pediatricians?

Andrzej Stawarski1,A,E,F, Paweł Maleika1,B,C,D

1 Department and Clinic of Pediatrics, Gastroenterology and Nutrition, Wroclaw Medical University, Poland

Abstract

Neuroendocrine tumors (NET) of the gastrointestinal tract and pancreas are extremely rare in the pediatric population and limited data is available. In most cases, NET of the gastrointestinal tract in children are located in the appendix. Pancreatic NET are a small but partially distinct group of the gastrointestinal neuroendocrine neoplasms. The most common in this group are insulinomas; however, in some research, the gastrinoma type neoplasms are perceived to be most common in children. This study reviews the typical clinical presentation, appropriate diagnostics, staging, and treatment of these uncommon neoplasms. It is important to know the epidemiology and symptomatology in this age group despite the fact that the majority of physicians treating the youngest patients will never have to deal with it. This will facilitate an early diagnosis in case of symptoms that may suggest neuroendocrine cancer. It appears necessary to create harmonized recommendations regarding the diagnosis, treatment and post-treatment follow-up for pediatric patients.

Key words

neuroendocrine tumors, pediatric, gastroenterology

References (32)

  1. Johnson PRV. Gastroenteropancreatic neuroendocrine (carcinoid) tumors in children. Semin Pediatr Surg. 2014;23(2):91–95. doi:10.1053/j.sempedsurg.2014.03.007
  2. Kloppel G. Classification and pathology of gastroenteropancreatic neuroendocrine neoplasms. Endocr Relat Cancer. 2011;18(Suppl 1):S1–S16. doi:10.1530/erc-11-0013
  3. WHO Classification of Tumours of the Digestive System. IARC: Lyon 2017 (in press). http://publiczno-prywatni/554.
  4. Yao JC, Hassan M, Phan A. 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):3063–3072. doi:10.1200/JCO.2007.15.4377
  5. Parkes S, Muir K, Al Sheyyab M. Carcinoid tumors of the appendix in children 1957–1986: Incidence, treatment and outcome. Br J Surg. 1993;80(4):502–504. doi:10.1002/bjs.1800800433
  6. Navalkele P, O’Dorisio M, O’Dorisio TM. Neuroendocrine tumors in children and young adults: Incidence, survival, and prevalence in the United States. Pancreas. 2010;39(2):278. doi:10.1097/01.mpa.0000363933.98540.e2
  7. Diets IJ, Nagtegaal ID, Loeffen J, et al. Childhood neuroendocrine tumours: A descriptive study revealing clues for genetic predisposition. Br J Cancer. 2016;116(2):163–168. doi:10.1038/bjc.2016.408
  8. Modlin IM, Lye KD, Kidd M. A 5-decade analysis of 13,715 carcinoid tumors. Cancer. 2003;97(4):934–959. doi:10.1002/cncr.11105
  9. Corpron CA, Black CT, Herzog CE. A half century of experience with carcinoid tumors in children. Am J Surg Pathol. 1995;170(6):606–608. doi:10.1016/S0002-9610(99)80025-7
  10. Spunt SL, Pratt CB, Rao BN, et al. Childhood carcinoid tumors: The St Jude Children’s Research Hospital experience. J Pediatr Surg. 2000;35(9):1282–1286. doi:10.1053/jpsu.2000.9297
  11. Prommegger R, Obrist P, Ensinger C. Retrospective evaluation of carcinoid tumors of the appendix in children. World J Surg. 2002;26(12):1489–1492. doi:10.1007/s00268-002-6440-3
  12. Dall’Igna P, Ferrari A, Luzzatto C, et al. Carcinoid tumor of the appendix in childhood: The experience of two Italian institutions. J Pediatr Gastroenterol Nutr. 2005;40(2):216–219. doi:10.1097/00005176-200502000-00025
  13. Service FJ, McMahon MM, O’Brien PC. Functioning insulinoma: Incidence, recurrence, and long-term survival of patients. A 60-year study. Mayo Clin Proc. 1991;66(7):711–719. doi:10.1016/S0025-6196(12)62083-7
  14. Ehehalt F, Saeger HD, Schmidt CM. Neuroendocrine tumors of the pancreas. Oncologist. 2009;14(5):456–467. doi:10.1634/theoncologist.2008-0259
  15. Bhatti TR, Ganapathy K, Huppmann AR, et al. Histologic and molecular profile of pediatric insulinomas: Evidence of a paternal parent-of-origin effect. J Clin Endocrinol Metab. 2016;101(3):914–922. doi:10.1210/jc.2015-2914
  16. Kulkarni KP, Sergi C. Appendix carcinoids in childhood: Long-term experience at a single institution in Western Canada and systematic review. Pediatr Int. 2013;55(2):157–162. doi:10.1111/ped.12047
  17. Moertel CL, Weiland LH, Telander RL. Carcinoid tumor of the appendix in the first two decades of life. J Pediatr Surg. 1990;25(10):1073–1075. doi:10.1016/0022-3468(90)90221-T
  18. Ryden SE, Drake RM, Franciosi RA. Carcinoid tumors of the appendix in children. Cancer. 1975;36(4):1538–1542. doi:10.1002/1097-0142(197510)36:4<1538::AID-CNCR2820360448>3.0.CO;2-C
  19. Solcia E, Capella C, Klöppel G. Atlas of Tumor Pathology: Tumors of the Pancreas. Washington: DC: Armed Forces Institute of Pathology; 1997.
  20. Citak EC, Taşkinlar H, Arpaci RB, et al. Primary lymph node gastrinoma: A rare cause of abdominal pain in childhood. J Pediatr Hemat Oncol. 2013;35(5):394–398. doi:10.1097/MPH.0b013e318298de7e
  21. Falconi M, Bartsch DK, Eriksson B, et al. Barcelona Consensus Conference participants. ENETS Consensus Guidelines for the management of patients with digestive neuroendocrine neoplasms of the digestive system: Well-differentiated pancreatic non-functioning tumors. Neuroendocrinology. 2012;95(2):120–134. doi:10.1159/000335587
  22. Kos-Kudła B, Blicharz-Dorniak J, Strzelczyk J, et al. Diagnostic and therapeutic guidelines for gastro-entero-pancreatic neuroendocrine neoplasms (recommended by the Polish Network of Neuroendocrine Tumours). Endokrynol Pol. 2017;68(2):79–110. doi:10.5603/EP.2017.0015
  23. Kos-Kudła B, Hubalewska-Dydejczyk A, Kuśnierz K, et al. Pancreatic endocrine tumors: Management guidelines (recommended by the Polish Network of Neuroendocrine Tumors). Endokrynol Pol. 2017;68(2):169–197. doi:10.5603/EP.2017.2017
  24. Kumbasar B, Kamel IR, Tekes A. Imaging of neuroendocrine tumors: Accuracy of helical CT versus SRS. Abdom Imaging. 2004;29(6):696–702. doi:10.1007/s00261-003-0162-3
  25. Kamaoui I, De-Luca V, Ficarelli S. Value of CT enteroclysis in suspected small-bowel carcinoid tumors. AJR Am J Roentgenol. 2010;194(3):629–633. doi:10.2214/AJR.09.2760
  26. Khanna G, O’Dorisio SM, Menda Y. Gastroenteropancreatic neuroendocrine tumors in children and young adults. Pediatr Radiol. 2008;38(3):251–259. doi:10.1007/s00247-007-0564-4
  27. Delle Fave G, O’Toole D, Sundin A, et al. Vienna Consensus Conference participants. ENETS Consensus Guidelines Update for Gastroduodenal Neuroendocrine Neoplasms. Neuroendocrinology. 2016;103(2):119–124. doi:10.1159/000443168
  28. Njere I, Smith LL, Thurairasa D, et al. Systematic review and meta-analysis of appendiceal carcinoid tumors in children. Pediatr Blood Cancer. 2018;65(8):e27069. doi:10.1002/pbc.27069
  29. de Lambert G, Lardy H, Martelli H. Surgical management of neuroendocrine tumors of the appendix in children and adolescents: A retrospective French multicenter study of 114 cases. Pediatr Blood Cancer. 2016;63(4):598–603. doi:10.1002/pbc.25823
  30. Boudreaux JP, Klimstra DS, Hassan MM, et al. The NANETS consensus guideline for the diagnosis and management of neuroendocrine tumors: Well-differentiated neuroendocrine tumors of the jejunum, ileum, appendix, and cecum. Pancreas. 2010;39(6):753–766. doi:10.1097/MPA.0b013e3181ebb2a5
  31. Hoe FM, Thornton PS, Wanner LA. Clinical features and insulin regulation in infants with a syndrome of prolonged neonatal hyperinsulinism. J Pediatr. 2006;148(2):207–212. doi:10.1016/j.jpeds.2005.10.002
  32. Patton TJ. Pediatric Zollinger–Ellison Syndrome. Medscape. https://emedicine.medscape.com/article/932553-overview. Updated October 6, 2017. Accessed February 11, 2019.
© Wroclaw Medical University