Appendiceal Neuroendocrine Tumours in Childhood: Italian TREP Project

作者：Virgone Calogero^*; Cecchetto Giovanni; Alaggio Rita; Ferrari Andrea; Bisogno Gianni; Conte Massimo; Inserra Alessandro; Fagnani Anna M; Indolfi Paolo; Salfi Nunzio; Dall'Igna Patrizia

来源：Journal of Pediatric Gastroenterology and Nutrition, 2014, 58(3): 333-338.

DOI：10.1097/MPG.0000000000000217

• cancer of the appendix
• carcinoid tumours
• children tumours
• neuroendocrine tumours
• rare diseases

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.

• 出版日期2014-3