An aggressive giant dermatofibrosarcoma of the parotid gland
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 the cellular origin being fibroblastic, histiocytic, or neuroectodermal.

A 38 years old, women presented with slow-growing large right parotid mass. A total parotidectomy performed with auriculectomy and reconstruction using ALT flap. Diagnosis confirmed by pathology and immunohistochemistry.

Tumor recurred in 10 months, the second surgery with subsequent chemoradiotherapy performed. Patient initially treated with wide resection, 4 cycles of chemotherapy and postoperative radiotherapy 40 Gy, with the recurrence in 10 months. A second surgery was performed followed by radiotherapy. She is disease-free for more than two years under follow-up.

• Parotid gland or parotid region is a very rare site with few published case reports, shares the common features of trunk and extremities 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 reliable marker for difficult diagnostic cases.

Read in detail here:
Dr. Dr A●●●●h K●●●l and 2 other likes this