Myxofibrosarcoma landscape: diagnostic pitfalls, clinical management and future perspectives

Silvia Vanni, Alessandro De Vita, Lorena Gurrieri, Valentina Fausti, Giacomo Miserocchi, Chiara Spadazzi, Chiara Liverani, Claudia Cocchi, Chiara Calabrese, Alberto Bongiovanni, Nada Riva, Laura Mercatali, Federica Pieri, Roberto Casadei, Enrico Lucarelli, Toni Ibrahim

Research output: Contribution to journalReview articlepeer-review

1 Scopus citations

Abstract

Myxofibrosarcoma (MFS) is a common entity of adult soft tissue sarcomas (STS) characterized by a predilection of the extremities and a high local recurrence rate. Originally classified as a myxoid variant of malignant fibrous histiocytoma, this musculoskeletal tumor has been recognized since 2002 as a distinct histotype showing a spectrum of malignant fibroblastic lesions with myxoid stroma, pleomorphism and curvilinear vessels. Currently, the molecular pathogenesis of MFS is still poorly understood and its genomic profile exhibits a complex karyotype with a number of aberrations including amplifications, deletions and loss of function. The diagnosis is challenging due to the unavailability of specific immunohistochemical markers and is based on the analysis of cytomorphologic features. The mainstay of treatment for localized disease is represented by surgical resection, with (neo)-adjuvant radio- and chemotherapy. In the metastatic setting, chemotherapy represents the backbone of treatments, however its role is still controversial and the outcome is very poor. Recent advent of genomic profiling, targeted therapies and larger enrollment of patients in translational and clinical studies, have improved the understanding of biological behavior and clinical outcome of such a disease. This review will provide an overview of current diagnostic pitfalls and clinical management of MFS. Finally, a look at future directions will be discussed.

Original languageEnglish (US)
JournalTherapeutic Advances in Medical Oncology
Volume14
DOIs
StatePublished - 2022

Keywords

  • chemotherapy
  • musculoskeletal tumor
  • myxofibrosarcoma
  • soft tissue sarcoma
  • targeted therapy
  • translational models

ASJC Scopus subject areas

  • Oncology

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