Unexpected Clinical Outcome for Myxoinflammatory Fibroblastic Sarcoma, When Should They Be Considered High Grade?

Area of Science:

• Oncology
• Pathology
• Soft Tissue Tumors

Background:

• Myxoinflammatory fibroblastic sarcoma (MIFS) is a rare soft tissue tumor characterized by a myxoid stroma and a tendency for local recurrence.
• MIFS typically exhibits low metastatic potential and is generally considered a low-grade malignancy.

Observation:

• A 58-year-old woman presented with a rapidly growing mass on her calf, initially misdiagnosed as benign.
• Despite complete excision with negative margins and radiotherapy, the tumor recurred and metastasized to the lungs within 21 months.
• The patient ultimately died from a malignant pleural effusion secondary to metastatic MIFS.

Findings:

• This case demonstrates an aggressive, atypical presentation of MIFS with rapid progression and fatal metastatic disease.
• The risk factors for metastasis in high-risk MIFS lesions remain poorly defined.
• Therapeutic options for metastatic MIFS are limited, and mortality, though rare, can occur.

Implications:

• Early detection and a high index of suspicion are crucial for extremity lesions suspected to be MIFS.
• Screening for metastasis should be considered in cases of aggressive or recurrent MIFS.
• Further research into targeted therapies based on tumor genomics may improve outcomes for metastatic MIFS.