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Myxoid dermatofibrosarcoma protuberans: morphological, ultrastructural and immunohistochemical features
A Orlandi1, L Bianchi, L G Spagnoli
1Institute of Anatomic Pathology, Tor Vergata University, Rome, Italy. orlandi@utovrm.it
Journal of Cutaneous Pathology
|October 9, 1998
Summary
This study presents two rare cases of myxoid dermatofibrosarcoma protuberans, a distinct variant of fibrohistiocytic skin tumors. These tumors exhibit unique myxoid changes and require consideration in diagnosing skin neoplasms.
Area of Science:
- Dermatopathology
- Oncology
- Surgical Pathology
Background:
- Dermatofibrosarcoma protuberans (DFSP) is a rare skin cancer.
- Uncommon variants of DFSP require further characterization.
- Myxoid changes in DFSP are not well-documented.
Observation:
- Two cases of DFSP with significant myxoid changes were observed.
- Tumors presented as recurrent, multinodular cutaneous plaques.
- Histopathology revealed diffuse myxoid areas with scattered spindle cells.
Findings:
- Myxoid DFSP showed positive fibrohistiocytic markers and negative muscular, neural, or progenitor cell antigens.
- Lower mitotic figures and proliferation rates were noted in myxoid areas compared to typical DFSP.
- Ultrastructural analysis identified fibroblast-like cells with cytoplasmic vesicles containing glycosaminoglycans.
Implications:
- Myxoid DFSP represents a distinct variant of this fibrohistiocytic tumor.
- Recognition of myxoid DFSP is crucial for accurate diagnosis.
- This variant should be included in the differential diagnosis of cutaneous myxoid neoplasms.