Pancreatic tumors in children and adolescents: the Italian TREP project experience.
BACKGROUND
Malignant pancreatic tumors are exceedingly rare in pediatric age and their clinical features and treatment usually go unappreciated by most pediatric oncologists and surgeons.


METHODS
From January 2000 to July 2009, 21 patients <18 years old with pancreatic tumors were prospectively registered in the Italian cooperative TREP project dedicated to very rare pediatric tumors.


RESULTS
Tumor types were 4 pancreatoblastomas, 2 pancreatic carcinomas, 3 neoplasms of the endocrine pancreas, and 12 solid pseudopapillary tumors. Three of the four patients with pancreatoblastoma had advanced disease at diagnosis and were given chemotherapy; at the time of this report, three patients were alive in first remission, while one died due to treatment toxicity. Both the cases of pancreatic carcinoma had the acinar cell subtype and successfully underwent pancreaticoduodenectomy with complete tumor resection, remaining without evidence of disease at the time of this analysis. The histological diagnoses of the three endocrine tumors were a malignant islet cell tumor, a gastrinoma, and a well-differentiated tumor. All 12 patients with solid pseudopapillary tumors underwent complete tumor resection and were given no adjuvant treatment; 11 were alive in first remission, while one experienced a local and distant relapse 5 years after diagnosis.


CONCLUSIONS
Surgery remains the keystone of treatment for pancreatic tumors in pediatric age as in adults. The TREP project shows that prospective cooperative studies are feasible even for such very rare tumors as these and may serve as a model for developing international cooperative schemes.
Dall'igna
Patrizia
P
Department of Pediatric, University Hospital of Padova, Padova, Italy.
Cecchetto
Giovanni
G
Bisogno
Gianni
G
Conte
Massimo
M
Chiesa
Pierluigi Lelli
PL
D'Angelo
Paolo
P
De Leonardis
Francesco
F
De Salvo
Gianluca
G
Favini
Francesca
F
Ferrari
Andrea
A
D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007558:Italy / Q000453:epidemiology; D008297:Male; D010190:Pancreatic Neoplasms / Q000453:epidemiology* / Q000473:pathology / Q000628:therapy; D011446:Prospective Studies; D035583:Rare Diseases / Q000453:epidemiology* / Q000473:pathology / Q000628:therapy; D016896:Treatment Outcome