Myxofibrosarcoma landscape: diagnostic pitfalls, clinical management and future perspectives

Author:

Vanni Silvia1,De Vita Alessandro2ORCID,Gurrieri Lorena1,Fausti Valentina1,Miserocchi Giacomo1,Spadazzi Chiara1,Liverani Chiara1,Cocchi Claudia1,Calabrese Chiara1,Bongiovanni Alberto1,Riva Nada1,Mercatali Laura1,Pieri Federica3,Casadei Roberto4,Lucarelli Enrico5,Ibrahim Toni5

Affiliation:

1. Osteoncology Unit, Bioscience Laboratory, IRCCS Istituto Romagnolo per lo Studio dei Tumori (IRST) ‘Dino Amadori’, Meldola, Italy

2. Osteoncology Unit, Bioscience Laboratory, IRCCS Istituto Romagnolo per lo Studio dei Tumori (IRST) ‘Dino Amadori’, Via P. Maroncelli 40, Meldola 47014, Forlì-Cesena, Italy

3. Pathology Unit, ‘Morgagni-Pierantoni’ Hospital, Forlì, Italy

4. Orthopedic Unit, ‘Morgagni-Pierantoni’ Hospital, Forlì, Italy

5. Osteoncology, Bone and Soft Tissue Sarcomas and Innovative Therapies Unit, IRCCS Istituto Ortopedico Rizzoli, Bologna, Italy

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.

Funder

Ministero della Salute

Publisher

SAGE Publications

Subject

Oncology

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