Relevance: Dermatofibrosarcoma protuberans (DFSP) is a cutaneous malignancy that arises from the dermis and invades deeper tissue. The cellular origin of DFSP is not clear. Evidence supports a fibroblastic, histiocytic, or neuroectodermal cellular origin. The article shares a clinical case of the treatment of aggressive dermatofibrosarcoma of the parotid salivary gland and describes the sequence of treatment and the treatment outcome. Results: A 38 years-old woman with a slow-growing large right parotid mass underwent a total parotidectomy with auriculectomy and reconstruction using ALT flap. The surgery in the scope of a wide resection was followed by 4 cycles of chemotherapy and postoperative radiotherapy up to 40 Gy. The tumor recurrence after 10 months was treated by the second surgery followed by chemoradiotherapy. No new recurrence was observed in the following two years. Conclusion: In the observed case, the giant tumor in the parotid salivary gland recurred after the initial surgery and adjuvant treatment. A tumor in the parotid region can recur despite aggressive initial treatment with wide resection and chemoradiotherapy.

HighlightsThe parotid region is a very rare site with few published case reports, shares the common features of trunk DFSP.Radiotherapy or chemoradiotherapy can be applied to large and recurrent cases, but with unclear benefit.Dermatofibrosarcoma protuberans is a rare tumor, with infiltrative margins, high local recurrence rate, and rare distant metastasis.CD34 immunohistochemistry is a reliable marker for difficult diagnostic cases.