Download PDF
Eur J Pediatr Surg 2019; 29(05): 449-457
DOI: 10.1055/s-0038-1667032
Original Article
Georg Thieme Verlag KG Stuttgart · New York

A National Long-Term Study of Neuroendocrine Tumors of the Appendix in Children: Are We Too Aggressive?

Christelle Sommer
1   Department of Pediatric Surgery, Hopitaux Universitaires de Geneve Hopital des Enfants, Geneva, Switzerland
,
Fabienne Gumy Pause
2   Department of Pediatric Hemato-oncology, Hopitaux Universitaires de Geneve Hopital des Enfants, Geneva, Switzerland
,
Manuel Diezi
3   Unit of Pediatric Hemato-oncology, Centre Hospitalier Universitaire Vaudois, Lausanne, Canton of Vaud, Switzerland
,
Anne-Laure Rougemont
4   Division of Clinical Pathology, Hopitaux Universitaires de Geneve, Geneva University Hospitals, Geneva, Switzerland
,
Barbara E. Wildhaber
1   Department of Pediatric Surgery, Hopitaux Universitaires de Geneve Hopital des Enfants, Geneva, Switzerland
› Author Affiliations
Further Information

Publication History

19 April 2018

04 June 2018

Publication Date:
01 August 2018 (online)

    Permissions and Reprints

Abstract

Introduction Pediatric neuroendocrine tumors (NETs) of the appendix are mostly detected incidentally after appendectomy for acute appendicitis. NET management is a matter of controversy. In this national, multicenter study, we aimed to establish guidelines based on our results and the literature.

Materials and Methods Medical records of children (0–16 years) with NET of the appendix, treated in Switzerland (1991–2012), were reviewed.

Results Forty cases (28 girls) were analyzed. Median age at diagnosis was 12.7 years (interquartile range [IQR]: 4.0). Tumor size was 0.1–24 mm (median: 0.6, IQR: 0.6). Four patients (10%) underwent additional surgery because of either tumor size > 15 mm (1/4), extension to the mesoappendix (1/4), or incomplete resection (2/4). Three patients with a tumor of ≥ 20 mm had no additional surgery. No patient had lymph node metastases. All patients were in complete remission at the last follow-up (median: 3.0 years, IQR: 10.9).

Conclusion We conclude from this study and from an extensive review of the literature that two criteria may point to the need for additional surgery, i.e., the possibility of regional lymph node involvement: tumor size > 20 mm and incomplete surgical resection margins. Childhood NET of the appendix has an excellent prognosis.

Keywords

neuroendocrine tumor - carcinoid - appendix - children
 

  • References

  • 1 WHO. Available at: http://apps.who.int/iris/bitstream/handle/10665/96612/9789241548496_%20eng.pdf?sequence=1&ua=1
    PubMed
  • 2 Johnson PRV. Gastroenteropancreatic neuroendocrine (carcinoid) tumors in children. Semin Pediatr Surg 2014; 23 (02) 91-95
    CrossrefPubMedGoogle Scholar
  • 3 Bosman FT, Carneiro F, Hruban RH, Theise ND. World Health Organization Classification of Tumours of the Digestive System, Pathology and Genetics of Tumours of the Digestive System. 4th ed. Lyon: IARC Press; 2010: 126
    Google Scholar
  • 4 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 (06) 753-766
    CrossrefPubMedGoogle Scholar
  • 5 Amin MB, Edge S, Greene F. , et al , eds. AJCC Cancer Staging Manual, 8th edition. Switzerland: Springer; 2016: 389-394
    Google Scholar
  • 6 Pape U-F, Niederle B, Costa F. , et al. ENETS Consensus Guidelines for Neuroendocrine Neoplasms of the Appendix (Excluding Goblet Cell Carcinomas). Neuroendocrinology 2016; 103 (02) 144-152
    CrossrefPubMedGoogle Scholar
  • 7 Boxberger N, Redlich A, Böger C. , et al. Neuroendocrine tumors of the appendix in children and adolescents. Pediatr Blood Cancer 2013; 60 (01) 65-70
    CrossrefPubMedGoogle Scholar
  • 8 Mathur A, Steffensen TS, Paidas CN, Ogera P, Kayton ML. The perforated appendiceal carcinoid in children: a surgical dilemma. J Pediatr Surg 2012; 47 (06) 1155-1158
    CrossrefPubMedGoogle Scholar
  • 9 Henderson L, Fehily C, Folaranmi S. , et al. Management and outcome of neuroendocrine tumours of the appendix-a two centre UK experience. J Pediatr Surg 2014; 49 (10) 1513-1517
    CrossrefPubMedGoogle Scholar
  • 10 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 (06) 606-608
    CrossrefPubMedGoogle Scholar
  • 11 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 (05) 622-625
    CrossrefPubMedGoogle Scholar
  • 12 Virgone C, Cecchetto G, Alaggio R. , et al. Appendiceal neuroendocrine tumors in childhood: Italian Trep Project. J Pediatr Gastroenterol Nutr 2014; 58 (03) 333-338
    CrossrefPubMedGoogle Scholar
  • 13 Doede T, Foss HD, Waldschmidt J. Carcinoid tumors of the appendix in children--epidemiology, clinical aspects and procedure. Eur J Pediatr Surg 2000; 10 (06) 372-377
    Article in Thieme ConnectPubMedGoogle Scholar
  • 14 de Lambert G, Lardy H, Martelli H, Orbach D, Gauthier F, Guérin F. 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 (04) 598-603
    CrossrefPubMedGoogle Scholar
  • 15 Redlich A, Boxberger N, Vorwerk P. Neuroendocrine Tumors of the Appendix, Rare Tumors in Children and Adolescents, Pediatric Oncology. Berlin, Germany: Springer Verlag; 2012: 277-278
    Google Scholar
  • 16 Wu H, Chintagumpala M, Hicks J, Nuchtern JG, Okcu MF, Venkatramani R. Neuroendocrine tumor of the appendix in children. J Pediatr Hematol Oncol 2017; 39 (02) 97-102
    CrossrefPubMedGoogle Scholar
  • 17 Kim SS, Kays DW, Larson SD, Islam S. Appendiceal carcinoids in children--management and outcomes. J Surg Res 2014; 192 (02) 250-253
    CrossrefPubMedGoogle Scholar
  • 18 Pérez-Albert P, de Rojas T, Lendínez MÁ. , et al. Management and outcome of children with neuroendocrine tumors of the appendix in Spain: is there room for improvement?. Clin Transl Oncol 2017; 19 (09) 1168-1172
    CrossrefPubMedGoogle Scholar
  • 19 Cernaianu G, Tannapfel A, Nounla J, Gonzalez-Vasquez R, Wiesel T, Tröbs R-B. Appendiceal carcinoid tumor with lymph node metastasis in a child: case report and review of the literature. J Pediatr Surg 2010; 45 (11) e1-e5
    PubMedGoogle Scholar
  • 20 Andersson A, Bergdahl L. Carcinoid tumors of the appendix in children. A report of 25 cases. Acta Chir Scand 1977; 143 (03) 173-175
    PubMedGoogle Scholar
  • 21 Volpe A, Willert J, Ihnken K, Treynor E, Moss RL. Metastatic appendiceal carcinoid tumor in a child. Med Pediatr Oncol 2000; 34 (03) 218-220
    CrossrefPubMedGoogle Scholar
  • 22 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 (02) 157-162
    CrossrefPubMedGoogle Scholar
  • 23 Lyons RF, Irfan M, Waldron R. , et al. Malignant neuroendocrine tumour of the appendix in childhood with loco-regional lymph node invasion. Diagn Pathol 2015; 10: 55
    CrossrefPubMedGoogle Scholar
  • 24 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 (02) 216-219
    CrossrefPubMedGoogle Scholar
  • 25 Vandevelde A, Gera P. Carcinoid tumours of the appendix in children having appendicectomies at Princess Margaret Hospital since 1995. J Pediatr Surg 2015; 50 (09) 1595-1599
    CrossrefPubMedGoogle Scholar
  • 26 Scott A, Upadhyay V. Carcinoid tumours of the appendix in children in Auckland, New Zealand: 1965-2008. N Z Med J 2011; 124 (1331): 56-60
    PubMedGoogle Scholar
  • 27 Ramage JK, Ahmed A, Ardill J. , et al. Guidelines for the management of gastroenteropancreatic neuroendocrine (including carcinoid) tumours (NETs). Gut 2012; 61 (01) 6-32
    CrossrefPubMedGoogle Scholar
  • 28 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) 1489-1492
    CrossrefPubMedGoogle Scholar