PubMed · 7930006
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L M Dzubow. 1994. Dermatofibrosarcoma protuberans.. https://doi.org/10.1111/j.1524-4725.1994.tb00443.x
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Cytogenetic analysis of an aggressive giant cell fibroblastoma in a 15-month-old male revealed the following balanced translocation: t(17;22)(q21.33;q13.1). This is the first cytogenetic report on this early childhood tumor.
We report a patient with unusual high grade dermatofibrosarcoma protuberans, confirmed by immunohistochemistry, which developed at radiotherapy field margins 5.7 years after radiotherapy for a squamous cell glottic cancer. The sarcoma relapsed locally after inadequate excision and developed lung metastases, as confirmed by fine needle aspiration. Radiotherapy induced soft tissue sarcomas may differ histologically from their spontaneous counterparts. Even a low radiation dose at the field margins can induce a soft tissue sarcoma.
Dermatofibrosarcoma protuberans (DFSP) is a slowly growing tumor that occurs in the dermis, and it has a tendency to recur repeatedly. We reported a case, 50-year-old woman, who underwent chest wall reconstruction due to recurrence of DFSP. Since the first operation for the tumor 35 years ago, she had undergone surgical procedures 30 times. We had to resect parts of the rib cartilages and the sternum because we suspected the tumor had reached to the anterior mediastinum. There has been no recurrence for 8 months since this operation. Importance of extensive resection of the tumor to prevent a recurrence and long term follow-up should be stress.