From the Pediatric Oncology Unit, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy
Author's disclosure of potential conflicts of interest are found at the end of this article.
Address reprint requests to Andrea Ferrari, MD, Pediatric Oncology Unit, Fondazione IRCCS Istituto Nazionale dei Tumori, Via G. Venezian, 1-20133 Milan, Italy; e-mail: andrea.ferrari{at}istitutotumori.mi.it
Overview: The Tumori Rari in Età Pediatrica (TREP) project is a national-scale cooperative project focusing on rare pediatric tumors. It was launched in Italy in 2000 with a goal of improving the clinical management and expanding the basic research currently available regarding the most uncommon pediatric cancers, which are defined as solid malignancies of childhood with an annual incidence of less than two per 1 million that are not included as part of other clinical trials. The project involved developing diagnostic and therapeutic recommendations for each of these rare tumors within the same framework and creating a network to ensure cooperation with other specialists (including medical oncologists) concerned with managing such diseases. Pathologic and biologic studies were also established. Since the TREP project began, it has enrolled 457 patients younger than age 18 from 36 Italian centers (at a rate of 54 patients/year). Thyroid carcinoma proved the most common histotype, followed by carcinoid tumors, skin tumors, gonadal nongerm-cell tumors, and nasopharyngeal carcinoma. When we compared the number of cases actually registered with the number of cases predicted in light of incidence data from the Italian population-based cancer registries, the vast majority of patients younger than age 15 were registered and treated according to the TREP guidelines; underreporting for the older adolescent age group was statistically significant. These observations suggest that international studies are warranted and feasible.
