TY - JOUR
T1 - Gastrointestinal tract carcinoma in pediatric and adolescent age
T2 - The Italian TREP project experience
AU - Indini, Alice
AU - Bisogno, Gianni
AU - Cecchetto, Giovanni
AU - Vitellaro, Marco
AU - Signoroni, Stefano
AU - Massimino, Maura
AU - Riccipetitoni, Giovanna
AU - Zecca, Marco
AU - Dall'Igna, Patrizia
AU - Debora De Pasquale, Maria
AU - Inserra, Alessandro
AU - Chiaravalli, Stefano
AU - Basso, Eleonora
AU - Virgone, Calogero
AU - Sorbara, Silvia
AU - Di Bartolomeo, Maria
AU - D'Angelo, Paolo
AU - Ferrari, Andrea
PY - 2017/12/1
Y1 - 2017/12/1
N2 - Background: Gastrointestinal (GI) carcinomas are very rare in the pediatric and adolescent age range. We report the clinical features, treatment, and outcome of a series of children and adolescents with GI carcinoma prospectively registered in the Italian Tumori Rari in Età Pediatrica (TREP) project. Methods: The TREP project developed diagnostic and therapeutic guidelines based on recommendations currently in use for adults. Clinical data were centrally registered and reviewed. Results: Fifteen patients were registered over the years 2000–2016. Most of the tumors were colorectal carcinomas (12 cases). All but one patient had advanced-stage disease (American Joint Committee on Cancer stages III–IV), and the majority of patients had aggressive histological subtypes, i.e. poorly differentiated (G3) (five patients), mucinous (four patients), and signet ring (two patients) adenocarcinomas. Surgery was performed in 13 of 15 patients, and was radical in nine of 13 patients. Only one patient received postoperative radiotherapy. All patients received chemotherapy, with the addition of bevacizumab in two cases. Nine patients were still alive at the time of the present report, but two of them had only just completed their treatment program and one patient is still on treatment. Six patients died due to disease progression. Conclusions: This prospective report on pediatric GI tract carcinomas confirms the rarity and biological aggressiveness of these diseases in pediatric and adolescent age. Further prospective studies are needed to explore the distinct biology of tumor in this age group in order to find new therapeutic targeted agents.
AB - Background: Gastrointestinal (GI) carcinomas are very rare in the pediatric and adolescent age range. We report the clinical features, treatment, and outcome of a series of children and adolescents with GI carcinoma prospectively registered in the Italian Tumori Rari in Età Pediatrica (TREP) project. Methods: The TREP project developed diagnostic and therapeutic guidelines based on recommendations currently in use for adults. Clinical data were centrally registered and reviewed. Results: Fifteen patients were registered over the years 2000–2016. Most of the tumors were colorectal carcinomas (12 cases). All but one patient had advanced-stage disease (American Joint Committee on Cancer stages III–IV), and the majority of patients had aggressive histological subtypes, i.e. poorly differentiated (G3) (five patients), mucinous (four patients), and signet ring (two patients) adenocarcinomas. Surgery was performed in 13 of 15 patients, and was radical in nine of 13 patients. Only one patient received postoperative radiotherapy. All patients received chemotherapy, with the addition of bevacizumab in two cases. Nine patients were still alive at the time of the present report, but two of them had only just completed their treatment program and one patient is still on treatment. Six patients died due to disease progression. Conclusions: This prospective report on pediatric GI tract carcinomas confirms the rarity and biological aggressiveness of these diseases in pediatric and adolescent age. Further prospective studies are needed to explore the distinct biology of tumor in this age group in order to find new therapeutic targeted agents.
KW - adenocarcinoma
KW - children
KW - colorectal cancer
KW - rare pediatric tumors
KW - TREP
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U2 - 10.1002/pbc.26658
DO - 10.1002/pbc.26658
M3 - Article
C2 - 28561949
AN - SCOPUS:85020041525
VL - 64
JO - Pediatric Blood and Cancer
JF - Pediatric Blood and Cancer
SN - 1545-5009
IS - 12
M1 - e26658
ER -