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Pigmented dermatofibrosarcoma protuberans (Bednar tumor). A pathologic, ultrastructural, and immunohistochemical
The American Journal of Surgical Pathology
|September 1, 1985
Summary
Pigmented dermatofibrosarcoma protuberans (DFSP) is a rare skin neoplasm, distinct from conventional DFSP by melanin-containing dendritic cells. Complete excision and close follow-up are recommended due to potential local recurrence.
Area of Science:
- Dermatopathology
- Oncology
- Surgical Pathology
Background:
- Pigmented dermatofibrosarcoma protuberans (DFSP) is a rare variant, representing 1-5% of DFSP cases.
- This neoplasm typically presents as an exophytic, multinodular lesion on the trunk, predominantly in individuals of Black ethnicity.
Purpose of the Study:
- To characterize the clinicopathological features of pigmented dermatofibrosarcoma protuberans.
- To differentiate pigmented DFSP from conventional DFSP and neurofibroma.
Main Methods:
- Microscopic examination of lesion morphology, including cellular arrangement and presence of dendritic cells.
- Electron microscopy to identify distinct cell populations.
- Review of clinical follow-up data for nine cases.
Main Results:
- Microscopically, pigmented DFSP shows spindled cells in a storiform pattern with melanin-containing dendritic cells, distinguishing it from conventional DFSP.
- Electron microscopy revealed three cell populations: fibroblast-like cells, cells with slender processes, and cells with melanosomes.
- Follow-up data indicated a potential for local recurrence, though distant metastases were not observed.
Conclusions:
- Pigmented DFSP is a distinct entity characterized by specific cellular features and melanin-containing dendritic cells.
- Despite controversy regarding its histogenesis, complete surgical excision and close monitoring are crucial due to its potential for local recurrence and probable intermediate malignant potential.