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Atypical Fibroxanthoma and Pleomorphic Dermal Sarcoma: Updates on Classification and Management.
Teo Soleymani1, Sumaira Z Aasi1, Roberto Novoa1
1Department of Dermatology, Stanford University School of Medicine, 450 Broadway Street, Pavilion C, 2nd Floor-MC5334, Redwood City, CA 94063, USA.
Dermatologic Clinics
|May 16, 2019
Summary
Atypical fibroxanthoma and pleomorphic dermal sarcoma are rare skin cancers. Distinguishing superficial tumors (atypical fibroxanthoma) from deep, high-risk tumors (pleomorphic dermal sarcoma) is key for effective treatment and patient outcomes.
Area of Science:
- Dermatology
- Surgical Pathology
- Oncology
Background:
- Atypical fibroxanthoma (AFX) and undifferentiated pleomorphic sarcoma (UPS), also known as pleomorphic dermal sarcoma (PDS), are rare malignant cutaneous neoplasms.
- These tumors exist on a clinicopathologic spectrum, sharing similarities but potentially differing in clinical behavior.
Purpose of the Study:
- To delineate the distinguishing clinicopathologic features of AFX and PDS.
- To correlate specific tumor characteristics with clinical behavior and patient outcomes.
Main Methods:
- Review of clinicopathologic features of AFX and PDS.
- Histologic evaluation focusing on tumor depth and high-risk features.
- Correlation of findings with clinical presentation and outcomes.
Main Results:
- Superficial tumors with minimal high-risk histologic features are characteristic of AFX.
- Deeper tumors exhibiting high-risk histologic features are indicative of PDS and associated with aggressive clinical behavior.
Conclusions:
- Recognition of distinguishing features between AFX and PDS is crucial for predicting clinical behavior.
- Surgical management with complete margin control is essential for both entities.
- High-risk tumors (PDS) necessitate comprehensive evaluation and multidisciplinary management for optimal outcomes.
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