Biology and Management of High-Grade Myxofibrosarcoma: State of the Art and Future Perspectives.

ABSTRACT: Myxofibrosarcoma (MFS) is one of the most common adult soft tissue sarcomas, typically arising in the extremities. Histologically, MFS is classified into three grades: low, intermediate, and high. Histological grades correlate with distant metastases and tumor-associated mortality. The diagnosis of MFS is challenging due to a lack of well-characterized immunohistochemical markers. High-grade MFS displays highly complex karyotypes with multiple copy number alterations. Recent integrated genomic studies have shown the predominance of somatic copy number aberrations. However, the molecular pathogenesis of high-grade MFS remains poorly understood. The standard treatment for localized MFS is surgical resection. The systemic treatment options for advanced disease are limited. This review provide

SUBMITTER: Nishio J 

PROVIDER: S-EPMC10572210 | biostudies-literature | 2023 Sep

REPOSITORIES: biostudies-literature