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Vol 14, No 6 (2018)
Review paper
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Malignant peripheral nerve sheath tumour (MPNST)

Anna M. Czarnecka, Paweł Sobczuk, Marcin Zdzienicki, Mateusz Spałek, Piotr Rutkowski
DOI: 10.5603/OCP.2018.0050
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Oncol Clin Pract 2018;14(6):364-376.

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Vol 14, No 6 (2018)
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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.

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.

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Keywords

malignant peripheral nerve sheath tumour; MPNST; Recklinghausen syndrome; NF1, sarcoma

About this article
Title

Malignant peripheral nerve sheath tumour (MPNST)

Journal

Oncology in Clinical Practice

Issue

Vol 14, No 6 (2018)

Article type

Review paper

Pages

364-376

DOI

10.5603/OCP.2018.0050

Bibliographic record

Oncol Clin Pract 2018;14(6):364-376.

Keywords

malignant peripheral nerve sheath tumour
MPNST
Recklinghausen syndrome
NF1
sarcoma

Authors

Anna M. Czarnecka
Paweł Sobczuk
Marcin Zdzienicki
Mateusz Spałek
Piotr Rutkowski

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