Myxofibrosarcoma landscape: diagnostic pitfalls, clinical management and future perspectives

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...

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Published in:Therapeutic Advances in Medical Oncology 2022-06, Vol.14, p.17588359221093973-17588359221093973
Main Authors: Vanni, Silvia, De Vita, Alessandro, Gurrieri, Lorena, Fausti, Valentina, Miserocchi, Giacomo, Spadazzi, Chiara, Liverani, Chiara, Cocchi, Claudia, Calabrese, Chiara, Bongiovanni, Alberto, Riva, Nada, Mercatali, Laura, Pieri, Federica, Casadei, Roberto, Lucarelli, Enrico, Ibrahim, Toni
Format: Article
Language:English
Subjects:
Cancer
Chemotherapy
Genomics
Histiocytoma
Karyotypes
Metastases
Muscular system
Pleomorphism
Review
Sarcoma
Stroma
Tumors
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Summary: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.
ISSN:1758-8359
1758-8340
1758-8359
DOI:10.1177/17588359221093973