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Review
. 2022 Jul;31(3):419-430.
doi: 10.1016/j.soc.2022.03.006.

Management of Myxofibrosarcoma and Undifferentiated Pleomorphic Sarcoma

Aimee M Crago  1 , Kenneth Cardona  2 , Hanna Koseła-Paterczyk  3 , Piotr Rutkowski  4
Affiliations
Review

Management of Myxofibrosarcoma and Undifferentiated Pleomorphic Sarcoma

Aimee M Crago et al. Surg Oncol Clin N Am. 2022 Jul.

Abstract

Undifferentiated pleomorphic sarcoma (UPS) and myxofibrosarcoma (MFS) are genomically complex tumors commonly diagnosed in the extremities or trunk of elderly patients. They likely represent a spectrum of disease differentiated by myxoid stroma and curvilinear vessels observed in MFS but not in UPS. Limb-sparing surgery is the standard of care although the infiltrative nature of MFS mandates wider resection margins than are necessary for UPS. UPS are conversely associated with high risks of distal recurrence, often prompting recommendations for adjuvant chemotherapy. In both histologies, anthracycline-based therapies or gemcitabine and docetaxel are used to manage advanced disease; immunotherapy may be of benefit in a subset of patients.

Keywords: Limb-sparing surgery; Malignant fibrous histiocytoma; Myxofibrosarcoma; Soft tissue sarcoma; Undifferentiated pleomorphic sarcoma; systemic therapy.

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Figures

Fig. 1.
Fig. 1.
Histologic classification of STS tumors diagnosed in patients admitted to MSKCC between 1982 and 2021. Liposarcoma, leiomyosarcoma, solitary fibrous tumor, and malignant peripheral nerve sheath tumor represent common histologies in the retroperitoneum and pelvis. UPS/MFH, myxofibrosarcoma, synovial sarcoma, and dermatofibrosarcoma protuberans represent a greater proportion of those tumors diagnosed in the extremity and trunk.
Fig. 2.
Fig. 2.
Gross and microscopic images depicting a representative (A) UPS and (B) MFS. The latter has a myxoid appearance on gross specimens, and thin, pale, myxoid stroma with curvilinear vessels. UPS is characterized by densely packed cells with large, pleomorphic nuclei, and numerous mitotic bodies.
Fig. 3.
Fig. 3.
Although both (A) UPS and (B) MFS seem similar on T1 sequences, MFS is hyperintense on T2 sequences and can have infiltrative, enhancing tails that extend outward from gross disease (arrow).
See this image and copyright information in PMC

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