Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2025 Aug 1;36(7):594-599.
doi: 10.1097/CAD.0000000000001722. Epub 2025 May 1.

Multimodal treatment with cisplatin for metastatic disease in malignant peripheral nerve sheath tumors: a case report and review

Rossella Hakim  1 , Maria Cristina Salone  2 , Giovanni Bozza  3 , Paolo Spinnato  4 , Alessandra Longhi  1
Affiliations
Review

Multimodal treatment with cisplatin for metastatic disease in malignant peripheral nerve sheath tumors: a case report and review

Rossella Hakim et al. Anticancer Drugs. .

Abstract

Malignant peripheral nerve sheath tumors (MPNSTs) are rare and have among the worst prognoses among all soft tissue sarcomas, with 5-year overall survival rates ranging from 16 to 52%. We report a case of a 50-year-old man with localized dorsal MPNST who developed local recurrence after 1 year and lung metastasis 3 years after diagnosis. He underwent primary tumor resection, two resections for local recurrence, one lung metastasectomy, seven lines of chemotherapy (epirubicin-ifosfamide, cisplatin-etoposide, trabectedin, pazopanib, carboplatin-etoposide, gemcitabine, and ifosfamide), and four courses of stereotactic body radiotherapy for lung metastases. The patient has shown long remission intervals and survival exceeding 7 years, with a good quality of life since the diagnosis of lung metastasis. We conducted a narrative review based on our treatment approach, considering the recent literature and drugs currently available for the treatment of MPNST.

Keywords: cisplatin; malignant peripheral nerve sheath tumor; neoadjuvant chemotherapy; stereotactic body radiotherapy.

PubMed Disclaimer

References

    1. Amirian ES, Goodman JC, New P, Scheurer ME. Pediatric and adult malignant peripheral nerve sheath tumors: an analysis of data from the surveillance, epidemiology, and end results program. J Neurooncol 2014; 116:609–616.
    1. Ferner RE, Gutmann DH. International consensus statement on malignant peripheral nerve sheath tumors in neurofibromatosis. Cancer Res 2002; 62:1573–1577.
    1. Foley KM, Woodruff JM, Ellis FT, Posner JB. Radiation-induced malignant and atypical peripheral nerve sheath tumors. Ann Neurol 1980; 7:311–318.
    1. Carli M, Ferrari A, Mattke A, Zanetti I, Casanova M, Bisogno G, et al. Pediatric malignant peripheral nerve sheath tumor: the Italian and German soft tissue sarcoma cooperative group. J Clin Oncol 2005; 23:8422–8430.
    1. The WHO Classification of Tumours Editorial Board. WHO classification of tumours soft tissue and bone tumours. 5th ed. IARC Press. 2020.

MeSH terms