Systemic Treatment for Advanced and Metastatic Malignant Peripheral Nerve Sheath Tumors-A Sarcoma Reference Center Experience.

ABSTRACT: Malignant peripheral nerve sheath tumor (MPNST) is a rare type of soft tissue sarcomas. The localized disease is usually treated with surgery along with perioperative chemo- or radiotherapy. However, up to 70% of patients can develop distant metastases. The study aimed to evaluate the modes and outcomes of systemic treatment of patients with diagnosed MPNST treated in a reference center. In total, 115 patients (56 female and 59 male) diagnosed with MPNST and treated due to unresectable or metastatic disease during 2000-2019 were included in the retrospective analysis. Schemes of systemic therapy and the outcomes-progression-free survival (PFS) and overall survival (OS)-were evaluated. The median PFS in the first line was 3.9 months (95% CI 2.5-5.4). Doxorubicin-based regimens were the most

SUBMITTER: Sobczuk P 

PROVIDER: S-EPMC7601777 | biostudies-literature | 2020 Sep

REPOSITORIES: biostudies-literature