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Dermatofibrosarcoma protuberans metastasizing to cavernous sinuses and lungs: a case report
1Department of Radiation Oncology, Hacettepe University Faculty of Medicine, 06100 Ankara, Turkey.
Japanese Journal of Clinical Oncology
|January 5, 2002
Summary
Dermatofibrosarcoma protuberans (DFSP), a rare skin cancer, can recur locally and metastasize. This case shows DFSP progressing to fibrosarcoma with metastasis to the brain and lungs.
Area of Science:
- Oncology
- Dermatology
- Pathology
Background:
- Dermatofibrosarcoma protuberans (DFSP) is a rare, locally aggressive skin cancer with low metastatic potential.
- Understanding DFSP progression is crucial for effective treatment and patient outcomes.
Observation:
- A 22-year-old female presented with a typical cutaneous DFSP.
- The patient experienced five local recurrences over eight years.
- Metastasis to the left cavernous sinus occurred in the eighth year, followed by right cavernous sinus and lung metastasis in the ninth year.
Findings:
- Each local recurrence demonstrated increased cellularity, higher mitotic index, and aggressive invasion.
- Metastatic tumors showed dedifferentiation from low-grade DFSP to high-grade fibrosarcoma.
- Immunohistochemistry confirmed CD34 positivity in all tumor specimens and p53 positivity in the metastatic tumor.
Implications:
- This case highlights the potential for DFSP to dedifferentiate and metastasize despite initial low-grade histology.
- The findings underscore the importance of vigilant monitoring for recurrence and metastasis in DFSP patients.
- Further research into the mechanisms of DFSP progression and metastasis is warranted.