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Wilms tumor, medulloblastoma, and rhabdomyosarcoma in adult patients: lessons learned from the pediatric experience

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Abstract

Wilms tumor (or nephroblastoma), rhabdomyosarcoma, and medulloblastoma, common embryonal tumors in children, can occasionally occur in adults, for whom survival is significantly inferior than pediatric patients. Available data on adults with Wilms tumor consist of case or case series reports. Among other factors, the unfamiliarity of adult oncologists and pathologists with nephroblastoma and consequent delays in initiating the appropriate risk-adapted chemotherapy may negatively influence outcomes. The survival decrement in adults with rhabdomyosarcoma has been attributed to the lack of centralized care, the inconsistent use of standard protocol-driven multimodal therapy, and lower chemotherapy tolerance in adult patients. In children with medulloblastoma, evidence from randomized clinical trials has led to risk-tailored therapies tuned on histology, extent of initial disease, and biological features. Such refinements are still missing for adults due to the lack of similar trials and studies that might provide the same or a different understanding regarding patients’ individual prognosis, treatment morbidity, and quality of life. Recent experiences have suggested that applying or adjusting pediatric protocols to adult patients with these tumors is feasible and can improve survival. Here, we provide an evaluation of the current evidence for the management of Wilms tumor, rhabdomyosarcoma, and medulloblastoma arising in adults. This review aims to promote the referral of adolescents and adults with pediatric tumors to pediatric centers for inclusion into pediatric protocols, or into protocols and studies specifically designed for that age group with the cooperation between pediatric and adult oncologists.

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Acknowledgments

We thank Associazione Bianca Garavaglia Onlus for their constant support.

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Authors and Affiliations

  1. Department of Medical Oncology and Hematology, Pediatric Oncology Unit, Fondazione IRCCS Istituto Nazionale dei Tumori, Via G. Venezian 1, 20133, Milan, Italy

    Filippo Spreafico, Andrea Ferrari, Veronica Biassoni, Elisabetta Schiavello, Giovanna Gattuso, Stefano Chiaravalli & Maura Massimino

  2. SOSD Oncologia Integrata del Giovane e Radioterapia Pediatrica, Centro di Riferimento Oncologico (CRO) IRCCS, Aviano, Italy

    Maurizio Mascarin

  3. Department of Diagnostic Pathology and Laboratory Medicine, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy

    Paola Collini

  4. Department of Radiology, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy

    Carlo Morosi

  5. Testis Surgery Unit, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy

    Davide Biasoni

  6. Pediatric Radiotherapy Unit, Radiation Oncology 1 Unit, Department of Radiation Oncology, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy

    Lorenza Gandola

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  1. Filippo Spreafico
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Spreafico, F., Ferrari, A., Mascarin, M. et al. Wilms tumor, medulloblastoma, and rhabdomyosarcoma in adult patients: lessons learned from the pediatric experience. Cancer Metastasis Rev 38, 683–694 (2019). https://doi.org/10.1007/s10555-019-09831-3

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