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[Dermatofibrosarcoma protuberans of the face. A case report]
M Bernal-Sprekelsen1, G H Rühl
1HNO-Univ.-Klinik Bochum, St.-Elisabeth-Hospital.
Laryngo- Rhino- Otologie
|January 1, 1991
Summary
This case report details a rare, slow-growing dermatofibrosarcoma protuberans (DFSP) on the face, treated over 40 years with multiple surgeries and radiotherapy due to recurrences. Metastases were observed, though rare in DFSP.
Area of Science:
- Oncology
- Dermatology
- Surgical Pathology
Background:
- Dermatofibrosarcoma protuberans (DFSP) is a rare, locally aggressive skin tumor.
- DFSP typically presents as a slow-growing, painless, flesh-colored dermal mass.
- Recurrence rates are high, but distant metastases are uncommon.
Observation:
- A 63-year-old woman presented with a facial dermatofibrosarcoma protuberans (DFSP).
- Over 40 years, 64 operations and radiotherapy were performed for recurrent DFSP.
- Two iatrogenic metastases were noted on the clavicula and breast following a Bakamjian flap procedure.
Findings:
- Histological examination revealed mature fibroblasts in bundles, exhibiting cart-wheel or storiform patterns with dermal infiltration.
- The tumor demonstrated slow growth and local invasiveness, characteristic of DFSP.
- Despite high local recurrence rates (>50%), distant metastases are exceptionally rare.
Implications:
- This extensive case highlights the challenges in managing recurrent DFSP, even with aggressive treatment.
- The occurrence of iatrogenic metastases warrants careful consideration of surgical techniques and drain site management.
- Long-term follow-up is crucial for patients with DFSP due to its propensity for recurrence.