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

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.