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Epithelioid Myxofibrosarcoma: Unveiling a Rare Variant's Progression and Therapeutic Challenges in an Elderly Patient
Mike Tran
McKenzie Clapp
Michael Subichin
Summa Health System
2024-02-01
Presenter: Mike Tran
Affidavit:
The entirety of the work on this project represents the original work of the resident.
Director Name: Ananth S Murthy
Author Category: Fellow Plastic Surgery
Presentation Category: Clinical
Abstract Category: General Reconstruction
Introduction:
Myxofibrosarcoma (MFS) is recently recognized as a distinct pathologic entity, comprising approximately 5% of soft tissue sarcomas. It has a diffusely infiltrative pattern and demonstrates a proclivity for local recurrence, with a 5-year survival of 60% to 77%. In this report, we present a case highlighting the epithelioid subtype-a rare variant of MFS that is indicative of a poor prognostic factor, underscoring the necessity for more aggressive treatment strategies.
Summary:
An 87-year-old male was discovered to have an enlarging lump on the right upper extremity following cardiac catheterization. Despite several surgical interventions for a presumed post-operative hematoma, which were performed due to concurrent dual antiplatelet therapy and anticoagulation, he continued to experience recalcitrant bleeding. Consequently, PRS consultation for radical debridement and soft tissue reconstruction was evoked. He presented to PRS clinic with a substantial mass, exhibiting central necrosis, distal sensory deficits, and active bleeding. An emergent radical excision was performed, revealing intraoperative findings of complete encasement of the brachial vessels and major plexus branches. Final pathology confirmed a high-grade epithelioid myxofibrosarcoma. Oncologic workup revealed extensive osseous metastatic disease, and the patient succumbed to the condition 6 months after the initial cardiac procedure.
Conclusion:
The epithelioid subtype of MFS is associated with poor 5-year survival rate (53.6%) and increased recurrence rate - multiple subsequent recurrences being commonplace. Recognition of this subtype warrants aggressive resection coupled with multi-modal adjuvant systemic therapy and radiation.
