title: Management of appendiceal carcinoid tumors in children.
abstract: BACKGROUND
  Appendiceal carcinoid tumors, also know as well-differentiated neuroendocrine neoplasms, are rare lesions detected incidentally after appendectomy in children. There are limited data about the natural history of these tumors, and guidelines regarding family counseling and need for additional surgery or follow-up imaging are not established in the pediatric age group. The purpose of this study was to review our institutional experience with appendiceal carcinoid tumors to provide data that might improve management.
  
  
  METHODS
  After institutional review board approval, the charts of all patients treated at our institution for an appendiceal carcinoid tumor between 2002 and 2014 were reviewed. Data collected included patient demographics, pathologic details, postoperative management, and follow-up information. Descriptive analyses were performed.
  
  
  RESULTS
  Twenty-eight patients were identified, which represents an incidence of 0.2% of children undergoing appendectomy during that time interval. The mean age at surgery was 13.8 ± 2.1 y; 54% were females. Two patients had symptoms suspicious for carcinoid syndrome at presentation, though none had evidence of metastatic disease. The mean tumor size was 0.73 ± 0.4 cm. Five patients (18%) underwent subsequent ileocecectomy or right hemicolectomy because of pathologic findings of invasion of the mesoappendix (n = 4) or lymphovascular invasion and subserosal extension (n = 1), two of whom had residual disease in the resected specimen (one in a lymph node). No recurrences have been detected at mean follow-up of 1.8 y.
  
  
  CONCLUSIONS
  Appendiceal carcinoid tumors are discovered incidentally in about 0.2% of children undergoing appendectomy. Based on findings from a large contemporary series, we can conclude that these tumors are generally small and demonstrate lymphovascular invasion or mesenteric extension in fewer than 20% of cases. Prospective, multicenter studies are necessary to better define the indication for ileocecectomy and follow-up imaging protocols.
authors.affiliation: Division of Pediatric Surgery, Michael E. DeBakey Department of Surgery, Baylor College of Medicine, Houston, Texas.
authors.forename: Sara C
authors.initials: SC
authors.lastname: Fallon
authors.affiliation: Department of Pathology, Texas Children's Hospital, Houston, Texas.
authors.forename: M John
authors.initials: MJ
authors.lastname: Hicks
authors.affiliation: Division of Pediatric Surgery, Michael E. DeBakey Department of Surgery, Baylor College of Medicine, Houston, Texas.
authors.forename: Jennifer L
authors.initials: JL
authors.lastname: Carpenter
authors.affiliation: Division of Pediatric Surgery, Michael E. DeBakey Department of Surgery, Baylor College of Medicine, Houston, Texas.
authors.forename: Sanjeev A
authors.initials: SA
authors.lastname: Vasudevan
authors.affiliation: Division of Pediatric Surgery, Michael E. DeBakey Department of Surgery, Baylor College of Medicine, Houston, Texas.
authors.forename: Jed G
authors.initials: JG
authors.lastname: Nuchtern
authors.affiliation: Division of Pediatric Surgery, Michael E. DeBakey Department of Surgery, Baylor College of Medicine, Houston, Texas. Electronic address: dlcass@texaschildrens.org.
authors.forename: Darrell L
authors.initials: DL
authors.lastname: Cass
mesh_terms: D000293:Adolescent; D001063:Appendiceal Neoplasms / Q000453:epidemiology* / Q000473:pathology / Q000628:therapy; D001065:Appendix / Q000473:pathology; D002276:Carcinoid Tumor / Q000453:epidemiology* / Q000473:pathology / Q000628:therapy; D002648:Child; D005260:Female; D006801:Humans; D015994:Incidence; D008297:Male; D012189:Retrospective Studies; D013781:Texas / Q000453:epidemiology
keywords: Appendicitis; Carcinoid syndrome; Carcinoid tumor; Hemicolectomy; Pediatric tumors