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[Pigmented dermatofibrosarcoma protuberance: a clinicopathologic analysis of 7 cases]
Jin Zhang1, Ru-song Zhang1, Xue Wei1
1Department of Pathology, Jinling Hospital, Nanjing 210002, China.
Zhonghua Bing Li Xue Za Zhi = Chinese Journal of Pathology
|February 11, 2014
Summary
Pigmented dermatofibrosarcoma protuberance (PDFSP) is a rare, intermediate-grade malignant tumor. Wide local excision is recommended to prevent recurrence, unlike simple excision.
Area of Science:
- Dermatopathology
- Surgical Oncology
- Medical Genetics
Background:
- Pigmented dermatofibrosarcoma protuberance (PDFSP) is a rare variant of dermatofibrosarcoma protuberans.
- Understanding its clinical and pathological features is crucial for accurate diagnosis and treatment.
Purpose of the Study:
- To investigate the clinical pathological features of PDFSP.
- To clarify diagnostic and differential diagnostic criteria for PDFSP.
- To analyze treatment outcomes and prognosis.
Main Methods:
- Analysis of clinical history, histopathology, and immunohistochemistry in seven PDFSP cases.
- Fluorescence in situ hybridization (FISH) to detect COL1A1/PDGFB fusion gene.
- Literature review.
Main Results:
- PDFSP presents as a spindle cell lesion with a storiform pattern and pigmented dendritic cells.
- Immunohistochemistry showed CD34 and vimentin positivity in spindle cells.
- FISH detected COL1A1/PDGFB fusion gene in 3 of 4 cases.
- Wide local excision showed no recurrence, while simple excision had recurrences.
Conclusions:
- PDFSP is a rare, intermediate-grade malignant tumor, a pigmented variant of DFSP.
- The origin of tumor cells remains debated.
- Awareness of PDFSP's histology is vital to avoid misdiagnosis as melanocytic neoplasms or tumors associated with neurocutaneous syndromes.
