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Vol 14, No 6 (2018)
Review paper
Published online: 2019-03-15

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Malignant peripheral nerve sheath tumour (MPNST)

Anna M. Czarnecka12, Paweł Sobczuk13, Marcin Zdzienicki1, Mateusz Spałek4, Piotr Rutkowski1
DOI: 10.5603/OCP.2018.0050
Oncol Clin Pract 2018;14(6):364-376.

Abstract

MPNST is a malignant neoplasm of peripheral nerves, usually arising in connection with nerve trunks of the limbs and torso. It can develop de novo or on the basis of an already existing neurofibroma. Such tumours constitute about 5% of soft tissue sarcomas. In 90%, they occur in patients in the 2–5 decade of life. The main risk factor for this cancer is type 1 neurofibromatosis (von Recklinghausen disease). The radical surgical treatment — tumour excision, within the limits of healthy tissues (wide local excision), combined with adjuvant radiotherapy, is of primary importance in the treatment of MPNST. In cases of metastatic disease, palliative chemotherapy is used, using doxorubicin or doxorubicin with ifosfamide. Clinical improvement after chemotherapy is observed in approximately 25–30% of patients. Considering the development of molecular biology research of MPNST, one can hope for development of inhibitors that show greater effectiveness than typical chemotherapy in these patients in the near future. Currently, clinical trials with pembrolizumab, nivolumab in combination with ipilimumab, pexidartinib (KIT inhibitor, CSF1R and FLT3) in combination with sirolimus, sapanisertib (TORC 1/2 inhibitor) or LOXO-195 (inhibitor of neurotrophic tyrosine kinase inhibitors NTRK type 1, 2 and 3) are performed in MNSNT patients.

Keywords: malignant peripheral nerve sheath tumourMPNSTRecklinghausen syndromeNF1sarcoma

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