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Appendiceal Neuroendocrine Tumours in Childhood: Italian TREP Project

Virgone, Calogero*; Cecchetto, Giovanni*; Alaggio, Rita; Ferrari, Andrea; Bisogno, Gianni§; Conte, Massimo||; Inserra, Alessandro; Fagnani, Anna M.#; Indolfi, Paolo**; Salfi, Nunzio††; Dall’Igna, Patrizia*

Journal of Pediatric Gastroenterology and Nutrition: March 2014 - Volume 58 - Issue 3 - p 333–338
doi: 10.1097/MPG.0000000000000217
Original Articles: Gastroenterology

Background: Neuroendocrine tumours (NETs) of the appendix are slow-growing tumours and, although rare, they are the most common gastrointestinal epithelial tumours in childhood and adolescence. The treatment and the follow-up screenings have not been standardised. In addition to this, although tumour size is considered the main prognostic variable to define the aggressiveness of approach, a precise cutoff needs to be established.

Methods: A total of 113 patients younger than 18 years with a diagnosis of appendiceal NETs were registered as of January 1, 2000, until May 30, 2013, within the Rare Tumors in Pediatric Age (TREP) project, an Italian multi-institutional network dedicated to rare tumours in children and adolescents. The recommendations of the Rare Tumors in Pediatric Age study included imaging and laboratory investigations. The treatment after appendectomy was decided on the basis of histology, tumour size, and imaging; primary reexcision (PRE) was not recommended in completely excised tumours, regardless of tumour size and invasiveness.

Results: A total of 113 of 113 patients had a diagnosis of well-differentiated NETs; in 108 of 113 the tumour was smaller than 2 cm and in 5, larger than 2 cm. Excision margins were free in 111 of 113 patients. In 3 of 113 a PRE was performed, and in 1 residual tumour was detected. All 113 of 113 patients are alive in complete remission (median follow-up of 41 months).

Conclusions: Reported data and our experience showed that no relapse or death occurred in children and adolescents affected by appendiceal NETs. Appendectomy alone should be considered curative for most patients, and a more aggressive surgical approach is warranted in the cases with incompletely excised tumours.

*Pediatric Surgery, Department of Women's and Children's Health

Pathology Unit, Department of Medical and Diagnostic Sciences and Special Therapies, University of Padua, Padua

Hematology Oncology, IRCCS Istituto Nazionale dei Tumori, Milan

§Hematology Oncology, Department of Women's and Children's Health, University-Hospital of Padua, Padua

||Hematology Oncology, Giannina Gaslini Children's Hospital, Genoa

Pediatric Surgery, Bambino Gesù Children's Hospital, Rome

#Pediatric Surgery, IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milan

**Hematology Oncology, Department of Pediatrics, II University, Naples

††Pathology Unit, Sant’Orsola-Malpighi Hospital, Bologna, Italy.

Address correspondence and reprint requests to Calogero Virgone, MD, Pediatric Surgery, Department of Women's and Children's Health, University Hospital, Via Giustiniani 3, 35128 Padua, Italy (e-mail:

Received 11 October, 2013

Accepted 11 October, 2013

The TREP project is partially supported by a grant from “Fondazione Cassa di Risparmio di Padova e Rovigo” and “Fondazione Citta’ della Speranza,” Padua.

The authors report no conflicts of interest.

© 2014 by European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology,