Skip to main content
SpringerLink
Log in

Appendiceal carcinoid tumor in children: implications for less radical surgery?

  • Review Article
  • Published:
Clinical and Translational Oncology Aims and scope Submit manuscript

Abstract

The discovery of an appendiceal carcinoid tumor found incidentally or during the course of diagnostic or therapeutic procedures is a burden to both the patient and clinician. The role of the correct surgical operation is paramount for lesions suspected to be malignant. In the pediatric population, appendiceal carcinoids continue to challenge the clinician in choosing the optimal treatment when lesions are larger than 2 cm or involve the appendical base. While the criteria used to define these distinct lesions are available in most cases, the management and treatment are still debated and controversial when considering more radical surgical intervention. The purpose of this article is to give an overview regarding the history, diagnosis, histopathology, management, and controversies associated with appendiceal carcinoid in the pediatric population.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

References

  1. Goede AC, Caplin ME, Winslet MC. Carcinoid tumour of the appendix. Br J Surg. 2003;90(11):1317–22 (review).

    Article  CAS  PubMed  Google Scholar 

  2. Boudreaux JP, Klimstra DS, Hassan MM, Woltering EA, Jensen RT, Goldsmith SJ, North American Neuroendocrine Tumor Society (NANETS), 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):753–66.

    Article  PubMed  Google Scholar 

  3. Modlin IM, Shapiro MD, Kidd M. Siegfried Oberndorfer: origins and perspectives of carcinoid tumors. Hum Pathol. 2004;35:1440–51.

    Article  PubMed  Google Scholar 

  4. Moertel CG, Weiland LH, Nagorney DM, Dockerty MB. Carcinoid tumor of the appendix: treatment and prognosis. N Engl J Med. 1987;317:1699–701.

    Article  CAS  PubMed  Google Scholar 

  5. Moertel CG, Dockerty MB, Judd ES. Carcinoid tumors of the vermiform appendix. Cancer. 1968;21(2):270–8.

    Article  CAS  PubMed  Google Scholar 

  6. Liu E, Telem DA, Hwang J, Warner RR, Dikman A, Divino CM. The clinical utility of Ki-67 in assessing tumor biology and aggressiveness in patients with appendiceal carcinoids. J Surg Oncol. 2010;102(4):338–41.

    Article  CAS  PubMed  Google Scholar 

  7. Prommegger R, Obrist P, Ensinger C, Profanter C, Mittermair R, Hager J. Retrospective evaluation of carcinoid tumors of the appendix in children. World J Surg. 2002;26(12):1489–92.

    Article  PubMed  Google Scholar 

  8. Mullen JT, Savarese DM. Carcinoid tumors of the appendix: a population-based study. J Surg Oncol. 2011;104:41–4.

    Article  PubMed  Google Scholar 

  9. Syracuse DC, Perzin KH, Price JB, Wiedel PD, Mesa-Tejada R. Carcinoid tumors of the appendix. Mesoappendiceal extension and nodal metastases. Ann Surg. 1979;190:58–63.

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  10. Williams ED, Sandler M. The classification of carcinoid tumours. Lancet. 1963;1(7275):238–9.

    Article  CAS  PubMed  Google Scholar 

  11. Corpron CA, Black CT, Herzog CE, Sellin RV, Lally KP, Andrassy RJ. A half century of experience with carcinoid tumors in children. Am J Surg. 1995;170(6):606–8.

    Article  CAS  PubMed  Google Scholar 

  12. Hatzipantelis E, Panagopoulou P, Sidi-Fragandrea V, Fragandrea I, Koliouskas DE. Carcinoid tumors of the appendix in children: experience from a tertiary center in northern Greece. J Pediatr Gastroenterol Nutr. 2010;51(5):622–5.

    Article  PubMed  Google Scholar 

  13. Tchana-Sato V, Detry O, Polus M, Thiry A, Detroz B, Maweja S, et al. Carcinoid tumor of the appendix: a consecutive series from 1237 appendectomies. World J Gastroenterol. 2006;12:6699–701.

    PubMed Central  PubMed  Google Scholar 

  14. Modlin IM, Sandor A. An analysis of 8305 cases of carcinoid tumors. Cancer. 1997;79(4):813–29.

    Article  CAS  PubMed  Google Scholar 

  15. Öberg K. Carcinoid tumors: current concepts in diagnosis and treatment. Oncologist. 1998;3(5):339–45.

    PubMed  Google Scholar 

  16. Spunt SL, Pratt CB, Rao BN, Pritchard M, Jenkins JJ, Hill DA, et al. Childhood carcinoid tumors: the St Jude Children’s Research Hospital experience. J Pediatr Surg. 2000;35(9):1282–6.

    Article  CAS  PubMed  Google Scholar 

  17. Safioleas MC, Moulakakis KG, Kontzoglou K, Stamoulis J, Nikou GC, Toubanakis C, et al. Carcinoid tumors of the appendix. Prognostic factors and evaluation of indications for right hemicolectomy. Hepatogastroenterology. 2005;52(61):123–7.

    PubMed  Google Scholar 

  18. Lundqvist M, Wilander E. Subepithelial neuroendocrine cells and carcinoid tumours of the human small intestine and appendix. A comparative immunohistochemical study with regard to serotonin, neuron-specific enolase and S-100 protein reactivity. J Pathol. 1986;148(2):141–7.

    Article  CAS  PubMed  Google Scholar 

  19. Klimstra DS, Modlin IR, Coppola D, Lloyd RV, Suster S. The pathologic classification of neuroendocrine tumors: a review of nomenclature, grading, and staging systems. Pancreas. 2010;39(6):707–12 (review).

    Article  PubMed  Google Scholar 

  20. 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):157–62.

    Article  PubMed  Google Scholar 

  21. Pinchot SN, Holen K, Sippel RS, Chen H. Carcinoid tumors. Oncologist. 2008;13(12):1255–69 (review).

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  22. Mathur A, Steffensen TS, Paidas CN, Ogera P. Kayton ML. The perforated appendiceal carcinoid in children: a surgical dilemma. J Pediatr Surg. 2012;47(6):1155–8 (review).

    Article  PubMed  Google Scholar 

  23. Anderson JR, Wilson BG. Carcinoid tumours of the appendix. Br J Surg. 1985;72(7):545–6.

    Article  CAS  PubMed  Google Scholar 

  24. O’Donnell ME, Carson J, Garstin WI. Surgical treatment of malignant carcinoid tumours of the appendix. Int J Clin Pract. 2007;61(3):431–7.

    Article  PubMed  Google Scholar 

  25. Stinner B, Rothmund M. Neuroendocrine tumours (carcinoids) of the appendix. Best Pract Res Clin Gastroenterol. 2005;19(5):729–38 (review).

    Article  CAS  PubMed  Google Scholar 

  26. Landry CS, Woodall C, Scoggins CR, McMasters KM, Martin RC 2nd. Analysis of 900 appendiceal carcinoid tumors for a proposed predictive staging system. Arch Surg. 2008;143(7):664–70 (discussion 670).

    Article  PubMed  Google Scholar 

  27. Warkel RL, Cooper PH, Helwig EB. Adenocarcinoid, a mucin-producing carcinoid tumor of the appendix: a study of 39 cases. Cancer. 1978;42(6):2781–93.

    Article  CAS  PubMed  Google Scholar 

  28. Tang LH. Epithelial neoplasms of the appendix. Arch Pathol Lab Med. 2010;134(11):1612–20.

    PubMed  Google Scholar 

  29. Auböck L, Höfler H. Extraepithelial intraneural endocrine cells as starting-points for gastrointestinal carcinoids. Virchows Arch A Pathol Anat Histopathol. 1983;401(1):17–33.

    Article  PubMed  Google Scholar 

  30. Abt AB, Carter SL. Goblet cell carcinoid of the appendix. An ultrastructural and histochemical study. Arch Pathol Lab Med. 1976;100(6):301–6.

    CAS  PubMed  Google Scholar 

  31. Bak M, Asschenfeldt P. Adenocarcinoid of the vermiform appendix. A clinicopathologic study of 20 cases. Dis Colon Rectum. 1988;31(8):605–12.

    Article  CAS  PubMed  Google Scholar 

  32. Burke AP, Sobin LH, Federspiel BH, Shekitka KM. Appendiceal carcinoids: correlation of histology and immunohistochemistry. Mod Pathol. 1989;2(6):630–7.

    CAS  PubMed  Google Scholar 

  33. Goddard MJ, Lonsdale RN. The histogenesis of appendiceal carcinoid tumours. Histopathology. 1992;20(4):345–9.

    Article  CAS  PubMed  Google Scholar 

  34. Burke AP, Sobin LH, Federspiel BH, Shekitka KM, Helwig EB. Goblet cell carcinoids and related tumors of the vermiform appendix. Am J Clin Pathol. 1990;94(1):27–35.

    CAS  PubMed  Google Scholar 

  35. Chetty R, Klimstra DS, Henson DE, Albores-Saavedra J. Combined classical carcinoid and goblet cell carcinoid tumor: a new morphologic variant of carcinoid tumor of the appendix. Am J Surg Pathol. 2010;34(8):1163–7.

    Article  PubMed  Google Scholar 

  36. Riddell RH, Petras RE, Williams GT, Sobin LH. Atlas of tumor pathology. Washington: AFIP; 2003. p. 304–9.

    Google Scholar 

  37. McCusker ME, Coté TR, Clegg LX, Sobin LH. Primary malignant neoplasms of the appendix: a population-based study from the surveillance, epidemiology and end-results program, 1973–1998. Cancer. 2002;94(12):3307–12.

    Article  PubMed  Google Scholar 

  38. Dimmler A, Geddert H, Faller G. EGFR, KRAS, BRAF-mutations and microsatellite instability are absent in goblet cell carcinoids of the appendix. Pathol Res Pract. 2014;210(5):274–8.

    Article  CAS  PubMed  Google Scholar 

  39. Kulke MH, Mayer RJ. Carcinoid tumors. N Engl J Med. 1999;340(11):858–68.

    Article  CAS  PubMed  Google Scholar 

  40. Volpe A, Willert J, Ihnken K, Treynor E, Moss RL. Metastatic appendiceal carcinoid tumor in a child. Med Pediatr Oncol. 2000;34(3):218–20 (review).

    Article  CAS  PubMed  Google Scholar 

  41. Andersson A, Bergdahl L. Carcinoid tumors of the appendix in children. A report of 25 cases. Acta Chir Scand. 1977;143(3):173–5.

    CAS  PubMed  Google Scholar 

  42. Cernaianu G, Tannapfel A, Nounla J, Gonzalez-Vasquez R, Wiesel T, Tröbs RB. Appendiceal carcinoid tumor with lymph node metastasis in a child: case report and review of the literature. J Pediatr Surg. 2010;45(11):e1–5.

    Article  PubMed  Google Scholar 

  43. National Comprehensive Cancer Institute. Carcinoid tumors. Available at: https://www.nccn.org. Accessed 22 May 2014.

  44. Arnold R, Chen YJ, Costa F, Falconi M, Gross D, Grossman AB, et al. Mallorca Consensus Conference participants; European Neuroendocrine Tumor Society, et al. ENETS Consensus guidelines for the standards of care in neuroendocrine tumors: follow-up and documentation. Neuroendocrinology. 2009;90(2):227–33.

    Article  CAS  PubMed  Google Scholar 

Download references

Acknowledgments

No funding was secured for this study.

Conflict of interest

The authors have no conflict of interest to disclose.

Author information

Authors and Affiliations

  1. Department of Surgery, St. Jude Children’s Research Hospital, MS 133, Room D3045, 262 Danny Thomas Place, Memphis, TN, 38105-3678, USA

    A. D. Malkan, F. N. Wahid, I. Fernandez-Pineda & J. A. Sandoval

Authors
  1. A. D. Malkan
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. F. N. Wahid
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. I. Fernandez-Pineda
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. J. A. Sandoval
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to J. A. Sandoval.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Malkan, A.D., Wahid, F.N., Fernandez-Pineda, I. et al. Appendiceal carcinoid tumor in children: implications for less radical surgery?. Clin Transl Oncol 17, 197–200 (2015). https://doi.org/10.1007/s12094-014-1196-4

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s12094-014-1196-4

Keywords

Search

Navigation