title: Management and outcome of children with neuroendocrine tumors of the appendix in Spain: Is there room for improvement?
abstract: OBJECTIVE
  Neuroendocrine tumors (NETs) are, after lymphomas, the most frequent gastrointestinal tumors in children, mainly located in the appendix. Best management remains unclear, given the absence of pediatric guidelines. We present the first Spanish series of pediatric patients with NETs.
  
  
  METHODS
  Retrospective study of all pediatric patients (<18 years) with NET treated in four oncology reference institutions in Spain between 1994 and 2015.
  
  
  RESULTS
  Seventeen patients were included. All patients presented with acute abdomen. TNM stage was T1a (82%) and T1b (12%). Extension study was heterogenous, with only 4 patients undergoing an OctreoScan. Four patients met criteria for second surgery (affected surgical margins or mesoappendix invasion), but it was only performed in two. Despite the diverse management, none of the patients relapsed during follow-up.
  
  
  CONCLUSIONS
  The disparity in diagnostic tests, second surgery criteria and follow-up shown in this study highlights the need for specific pediatric guidelines.
authors.affiliation: Pediatric Oncology and Hematology Department, Hospital Infantil Universitario Niño Jesús, Avenida Menéndez Pelayo, 65, 28009, Madrid, Spain.
authors.forename: P
authors.initials: P
authors.lastname: Pérez-Albert
authors.affiliation: Pediatric Oncology and Hematology Department, Hospital Infantil Universitario Niño Jesús, Avenida Menéndez Pelayo, 65, 28009, Madrid, Spain. teresa.rojas@salud.madrid.org.
authors.forename: T
authors.initials: T
authors.lastname: de Rojas
authors.affiliation: Pediatric Oncology and Hematology Department, Hospital Regional Universitario de Málaga, Málaga, Spain.
authors.forename: M Á
authors.initials: MÁ
authors.lastname: Lendínez
authors.affiliation: Pediatric Oncology and Hematology Department, Hospital Infantil Universitario Niño Jesús, Avenida Menéndez Pelayo, 65, 28009, Madrid, Spain.
authors.forename: L
authors.initials: L
authors.lastname: Illade
authors.affiliation: Pediatric Oncology and Hematology Department, Hospital Universitario Donostia, Donostia, Spain.
authors.forename: M
authors.initials: M
authors.lastname: García-Abos
authors.affiliation: Emergency Department, Hospital Infantil Universitario Niño Jesús, Madrid, Spain.
authors.forename: J
authors.initials: J
authors.lastname: Alonso-Cadenas
authors.affiliation: Pediatric Oncology and Hematology Department, Hospital Infantil Universitario Niño Jesús, Avenida Menéndez Pelayo, 65, 28009, Madrid, Spain.
authors.forename: M
authors.initials: M
authors.lastname: Andión
authors.affiliation: Pediatric Oncology and Hematology Department, Hospital Infantil Universitario Niño Jesús, Avenida Menéndez Pelayo, 65, 28009, Madrid, Spain.
authors.forename: L
authors.initials: L
authors.lastname: Madero
mesh_terms: D000293:Adolescent; D001063:Appendiceal Neoplasms / Q000175:diagnosis* / Q000473:pathology* / Q000601:surgery; D002648:Child; D005260:Female; D006801:Humans; D008297:Male; D018358:Neuroendocrine Tumors / Q000175:diagnosis* / Q000473:pathology* / Q000601:surgery; D012189:Retrospective Studies; D013030:Spain; D016896:Treatment Outcome
keywords: Appendix; Carcinoid tumors; Children; NET; Neuroendocrine tumors; OctreoScan