Sarcomas correspond to approximately 1 percent of all head and neck cancers. Dermatofibrosarcoma protuberans (DFSP) is a rare malignancy that accounts for 1 percent of all soft tissue sarcomas. The incidence of DFSP is 0.8 cases per 1 million persons. The tumor presents a low grade of malignancy with a tendency toward local recurrence, especially in the head and neck region, but rarely metastasizes. This behavior is probably related to certain histologic subtypes, as well as inadequate margins of resection.Surgical excision is the treatment of choice for DFSP. Adjuvant radiotherapy is used for cases in which adequate surgical margins cannot be obtained or in cases involving advanced tumors. The role of chemotherapy is still not well established, but advances in the understanding of the pathogenesis of DFSP have drawn attention to this therapeutic possibility.We report a case of a facial giant DFSP that required both a complex treatment and reconstruction. Because of the lesion's severe extension, several surgical interventions were required to achieve a reasonable result.
展开▼
