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Management of Childhood Malignant Peripheral Nerve Sheath Tumor

  • Therapy In Practice
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Abstract

Malignant peripheral nerve sheath tumor (MPNST) is rare, but is one of the most frequent non-rhabdomyosarcoma soft-tissue sarcomas in the pediatric population. These tumors occur most frequently at axial sites and are characterized by local aggressiveness and a propensity to metastasize. They are often associated with neurofibromatosis type 1 (NF-1): the lifetime risk of patients with NF-1 developing MPNST has been estimated at 8–13%, compared with 0.001% in the general population.

Because of the rarity of this tumor, little information is available on its clinical management, particularly in the pediatric age group. In a recent report on the clinical findings and treatment outcomes from a large number of children and adolescents with MPNST in an Italian and German series, less satisfactory overall outcomes than those for other pediatric sarcomas were described. Therefore, the approach to the treatment of patients with MPNST should be aggressive and risk adapted, and is necessarily complex. Patients should be referred to selected institutions with adequate experience in treating soft-tissue sarcomas, and with the multidisciplinary skills for enrolling patients in clinical trials.

Surgical resection represents the mainstay of treatment, while the role of adjuvant treatment is not yet clear. Post-operative radiotherapy seems to have a role in improving local control, although the potential morbidity of irradiation should be taken into account, particularly when treating children. Although lack of local control is the major cause of treatment failure, MPNST may give rise to distant metastases. These tumors are usually considered as having uncertain chemosensitivity, but recent evidence suggests that there may be a role for chemotherapy in patients with a high-grade histology.

For the near future, our hopes lie in the development of novel tailored therapies directed specifically against the molecular targets of the neoplastic cells: soft-tissue sarcomas seem particularly promising candidates for targeted therapy.

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Acknowledgements

No sources of funding were used to assist in the preparation of this review. The authors have no conflicts of interest that are directly relevant to the content of this review.

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Authors and Affiliations

  1. Pediatric Oncology Unit, Istituto Nazionale Tumori, Milan, Italy

    Andrea Ferrari

  2. Department of Pediatrics, Hematology Oncology Division, University Hospital of Padova, Via Giustiniani, 3, 35128, Padova PD, Italy

    Gianni Bisogno &  Modesto Carli

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  1. Andrea Ferrari
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Correspondence to Modesto Carli.

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Ferrari, A., Bisogno, G. & Carli, M. Management of Childhood Malignant Peripheral Nerve Sheath Tumor. Pediatr-Drugs 9, 239–248 (2007). https://doi.org/10.2165/00148581-200709040-00005

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