Aside from the CTNNB1 and adenomatous polyposis coli (APC) mutations, the genetic profile of pediatric aggressive fibromatosis (AF) has remained poorly characterized. The aim of this study was to shed more light on the mutational spectrum of pediatric AF, comparing it with its adult counterpart, with a view to identifying biomarkers for use as prognostic factors or new potential therapeutic targets. CTNNB1,APC,AKT1,BRAF TP53, and RET Sanger sequencing and next‐generation sequencing (NGS) with the 50‐gene Ion AmpliSeq Cancer Hotspot Panel v2 were performed on formalin‐fixed samples from 28 pediatric and 33 adult AFs. The prognostic value of CTNNB1,AKT1, and BRAF mutations in pediatric AF patients was investigated. Recurrence‐free survival (RFS) curves were estimated with the Kaplan–Meier method and statistical comparisons were drawn using the log‐rank test. In addition to the CTNNB1 mutation (64%), pediatric AF showed AKT1 (31%), BRAF (19%), and TP53 (9%) mutations, whereas only the CTNNB1 mutation was found in adult AF. The polymorphism Q472H VEGFR was identified in both pediatric (56%) and adult (40%) AF. Our results indicate that the mutational spectrum of pediatric AF is more complex than that of adult AF, with multiple gene mutations involving not only CTNNB1 but also AKT1 and BRAF. This intriguing finding may have clinical implications and warrants further investigations.
Aims and Background: Adolescent patients with cancer need psychological support in order to face the traumatic event of cancer diagnosis and to preserve continuity with their normal lives. Creative projects or laboratories may help young patients express their thoughts and feelings. Methods: The Youth Project developed activities dedicated to adolescents to give them a chance to vent their creative spirit and express themselves freely. In the first project, the teenagers designed their own fashion collection in all its various stages under the artistic direction of a well-known fashion designer, creating their own brand name (B.Live), and organized a fashion show. Results: In all, 24 patients from 15 to 20 years old took part in the project. The fashion project proved a fundamental resource in helping the young patients involved to regain a positive self-image and the feeling that they could take action, both on themselves and in their relations with others. Conclusions: Facilitating the experience of beauty may enable hope to withstand the anguish caused by disease. This experience integrated the usual forms of psychological support to offer patients a form of expression and support during the course of their treatment.
The Youth Project is a program developed at the Pediatric Oncology Unit at the Istituto Nazionale Tumori in Milan, dedicated to adolescents and young adults with cancer. Among its various goals, the Youth Project organizes structured creative activities with the support of professionals, with the objective of offering young people a new way to express their hopes and fears. This article describes a project centered around music: patients created a Christmas carol with the help of musicians and authors. The adolescents explained with their own words the meaning of the lyrics, telling the story of a Christmas spent in a cancer hospital ward.