Luca Bergamaschi

Clouds of Oxygen: Adolescents With Cancer Tell Their Story in Music

Andrea Ferrari, Michela Casanova, Stefano Chiaravalli, Chiara Magni, Roberto Luksch, Monica Terenziani, Filippo Spreafico, Daniela Polastri, Cristina Meazza, Serena Catania, Elisabetta Schiavello, Veronica Biassoni, Marta Podda, Luca Bergamaschi, Nadia Puma, Carla Moscheo, Giacomo Gotti, Maura Massimino, Fondazione IRCCS Istituto Nazionale Tumori; Laura Veneroni, Carlo Alfredo Clerici, University of Milan, Milan, Italy.

The Sooner the Better? How Symptom Interval Correlates With Outcome in Children and Adolescents With Solid Tumors: Regression Tree Analysis of the Findings of a Prospective Study

The potential impact of diagnostic delays on patients’ outcomes is a debated issue in pediatric oncology and discordant results have been published so far. We attempted to tackle this issue by analyzing a prospective series of 351 consecutive children and adolescents with solid malignancies using innovative statistical tools.

Measuring the efficacy of a project for adolescents and young adults with cancer: A study from the Milan Youth Project

Various projects dedicated specifically to adolescents and young adults (AYA) with cancer have been developed in recent years. A critical aspect of such programs is the ability to demonstrate its value, and therefore how to measure desired outcomes.

Anaplastic lymphoma kinase aberrations correlate with metastatic features in pediatric rhabdomyosarcoma

Rhabdomyosarcoma (RMS) is the most frequent soft tissue tumor in childhood and arises from immature mesenchymal cells committed to skeletal muscle differentiation. Anaplastic Lymphoma Kinase (ALK) is a receptor tyrosine kinase aberrantly expressed in several cancers. Moreover, ALK full-length receptor protein has been observed in RMS, although its clinical and functional significance is yet controversial. The role of ALK and its clinical relevance were investigated in a selected cohort of 74 FFPE pediatric RMS and a panel of RMS cell lines, evaluating its gene and protein status, utilizing Fluorescent In Situ Hybridization (FISH), immunohistochemistry (IHC) and Western blot approaches. Moreover, to get insight into its possible therapeutic relevance, effects of ALK silencing on cell proliferation, invasion and apoptosis were studied in RMS cells. ALK IHC positivity was significantly correlated with gene copy number gain, the alveolar subtype, PAX3/7-FOXO1 rearrangements, the presence of metastasis at diagnosis and a worse overall outcome. Furthermore, EML4-ALK fusion gene associated with higher protein expression was identified in an embryonal RMS. ALK silencing in RH30 ALK positive cells strongly inhibited invasion capability. Overall, our data suggest a potential role of ALK in pediatric RMS.

Salvage rates and prognostic factors after relapse in children and adolescents with malignant peripheral nerve sheath tumors

Malignant peripheral nerve sheath tumor (MPNST) is one of the most common nonrhabdomyosarcoma soft tissue sarcomas encountered in pediatric age, and it is generally characterized by poor outcome, particularly for relapsing patients.

Response to pazopanib in two pediatric patients with pretreated relapsing synovial sarcoma.

Pazopanib is an oral multikinase inhibitor that has proved effective in adults treated for relapsing soft tissue sarcoma and synovial sarcoma in particular. Two cases are reported here of pediatric patients with pretreated relapsing synovial sarcoma whose tumors showed a prolonged response to pazopanib given on compassionate grounds. These results suggest that new agents found effective in adult patients might achieve similar results in adolescents with the same disease. Facilitating the availability of new drugs for children and adolescents is a major challenge for pediatric oncologists.

PD-L1 assessment in pediatric rhabdomyosarcoma: a pilot study

BackgroundRhabdomyosarcomas (RMSs) are the most frequent soft tissue sarcoma in children and adolescents, defined by skeletal muscle differentiation and the status of FOXO1 fusions. In pediatric malignancies, in particular RMS, scant and controversial observations are reported about PD-L1 expression as a putative biomarker and few immune checkpoint clinical trials are conducted.MethodsPD-L1 assessment was evaluated by immunohistochemistry (IHC) utilizing two anti-PDL1 antibodies, in a pilot cohort of 25 RMS. Results were confirmed in primary and commercial RMS cell lines by cytofluorimetric analysis and IHC.ResultsPD-L1 expression was detectable, by both anti-PD-L1 antibodies, in the immune contexture of immune cells infiltrating and/or surrounding the tumor, in 15/25 (60%) RMS, while absent expression was observed in neoplastic cells. Flow cytometry analysis and PD-L1 IHC of commercial and primary RMS cell lines confirmed a very small percentage of PD-L1 positive-tumor cells, under the detection limits of conventional IHC. Interestingly, increased PD-L1 expression was observed in the immune contexture of 4 RMS cases post chemotherapy compared to their matched pre-treatment samples.ConclusionHere we identify a peculiar pattern of PD-L1 expression in our RMS series with scanty positive-tumor cells detected by flow cytometry, and recurrent expression in the immune cells surrounding or infiltrating the tumor burden.

Pediatric nonrhabdomyosarcoma soft tissue sarcomas arising at visceral sites

Pediatric nonrhabdomyosarcoma soft tissue sarcomas (NRSTS) may rarely occur in visceral tissues, and little is known about their clinical history. The present study retrospectively analyzed a group of patients prospectively registered in Italian pediatric protocols conducted between 1979 and 2004.

When curing a pediatric tumor is not enough: The case of a psychiatric disorder in a woman surviving osteosarcoma

Aims and background We describe the case of a woman cured of osteosarcoma who took part in a mono-institutional study using different questionnaires to assess pediatric cancer survivors’ quality of life and behavioral features 12 years after completing her cancer treatment. Results The high levels of psychological distress and psychopathologic symptoms revealed by this patient prompted us to offer her specific and prolonged support at our institution, since she refused to seek the help of other psychiatric services. The woman revealed a dysfunctional social and family setting and a borderline personality disorder. She was hospitalized after attempting suicide. No psychological distress had previously come to light during her long follow-up for cancer. Conclusions Cancer survivors are at risk of psychological and behavioral problems, so they should be followed up over time. Questionnaires and standard scales are important, but not enough: the physician-patient relationship is crucial to bring out a patients psychological issues and needs. This means that dedicated resources should be made available, whenever possible.