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Fibrosarcomatous dermatofibrosarcoma protuberans: a rapidly growing 30 cm mass on the posterior scalp
Bhoowit Lerttiendamrong1, Pavinee Annoppornchai1, Pasu Promniyom1
1Division of Plastic and Reconstructive Surgery, Department of Surgery, Faculty of Medicine, Chulalongkorn University, Bangkok 10330, Thailand.
Abstract:
Dermatofibrosarcoma protuberans (DFSP) is a rare, slow-growing sarcoma of the skin and subcutaneous tissue, accounting for around 5 cases per million per year. Fibrosarcomatous transformation of DFSP occurs in 10%-15% of DFSP cases, with a higher risk of local recurrence, metastasis, and death. We present a case of a male in his 30s with a complaint of rapidly progressive mass in the occipital region of the head. Within 1 year, the mass enlarged by >30 cm. Physical examination revealed a skin-colored 40×30 cm mass with an overlying skin necrosis at the posterior scalp. Brain, neck, and chest computed tomography (CT) scans were performed. The mass was surgically excised by wide excision with a 2 cm margin. Pathological report confirmed fibrosarcomatous DFSP Grade 3 with decreased CD34 expression. Delayed reconstruction of free flap and split-thickness skin graft were subsequently performed. No recurrence was detected 3 months postoperatively.
Insights
Dermatofibrosarcoma protuberans (DFSP) can transform into fibrosarcoma, posing a significant risk. This case highlights successful surgical management of a rapidly growing fibrosarcomatous DFSP.
Area of Science:
- Oncology
- Dermatopathology
Background:
- Dermatofibrosarcoma protuberans (DFSP) is a rare cutaneous sarcoma.
- Fibrosarcomatous transformation (FT) of DFSP increases recurrence and metastasis risk.
Observation:
- A rapidly enlarging, large occipital mass (>30 cm in 1 year) with skin necrosis was observed.
- Imaging confirmed a large mass in the posterior scalp.
Findings:
- Pathology revealed fibrosarcomatous DFSP Grade 3 with diminished CD34 expression.
- Surgical wide excision (2 cm margin) and subsequent flap reconstruction were performed.
Implications:
- Aggressive surgical management can be effective for advanced fibrosarcomatous DFSP.
- This case demonstrates successful treatment with no recurrence at 3 months post-surgery.
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