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Transformed dermatofibrosarcoma protuberans: a clinicopathological study of eight cases
1Department of Pathology, University of Debrecen, Medical and Health Science Centre, Debrecen, H-4012 Hungary. szollosi@jaguar.dote.hu
Journal of Clinical Pathology
|June 25, 2005
Summary
Transformed dermatofibrosarcoma protuberans (DFSP) with sarcomatous areas may be more aggressive. However, surgical treatment, particularly wide local excision, can influence tumor behavior and reduce recurrence risk.
Area of Science:
- Oncology
- Surgical Pathology
- Dermatopathology
Background:
- Dermatofibrosarcoma protuberans (DFSP) can undergo malignant transformation into fibrosarcoma (FS) or malignant fibrous histiocytoma (MFH).
- The biological behavior of transformed DFSPs, including risk of recurrence and metastasis, is debated.
- Previous studies have yielded conflicting results regarding the aggressiveness of transformed DFSPs compared to conventional DFSP.
Purpose of the Study:
- To investigate the clinicopathological features and biological behavior of transformed DFSPs.
- To evaluate the impact of surgical treatment on the outcomes of patients with transformed DFSP.
Main Methods:
- Clinicopathological analysis of eight transformed DFSP cases.
- Patient follow-up ranging from 4 to 36 years.
- Histopathological assessment of tumor characteristics, including sarcomatous component type and mitotic activity, and CD34 expression.
Main Results:
- Transformed DFSPs occurred on the trunk and lower extremity, with sarcomatous change presenting de novo.
- Fibrosarcoma (FS) and malignant fibrous histiocytoma (MFH) were the predominant transformation types.
- While some patients experienced recurrence and metastasis, wide local excision with negative margins was associated with a lower recurrence rate.
Conclusions:
- Transformed DFSP may exhibit more aggressive behavior than conventional DFSP.
- Surgical treatment, especially achieving histopathologically negative margins, significantly influences the clinical outcomes.
- Complete surgical resection is crucial for managing transformed DFSP and minimizing the risk of recurrence and metastasis.