dermatofibrosarcomas

Dermatofibrosarcoma protuberans (DFSP) is a rare, slow-growing soft tissue sarcoma that arises in the dermis, a deeper layer of the skin. It is considered a low-grade malignant tumor, meaning it grows slowly and rarely metastasizes to distant organs. However, DFSP has a high rate of local recurrence if not completely removed.

These tumors typically present as firm, flesh-colored to reddish-brown nodules or plaques, often on the trunk and extremities. They can be painless or mildly itchy. DFSP can be mistaken for benign skin conditions like dermatofibromas or keloids, leading to delayed diagnosis.

The exact cause of DFSP is unknown, but some studies suggest it may be associated with prior trauma or insect bites at the site of origin. It can occur at any age but is more common in adults between 30 and 50 years old, with a slight predilection for women.

Diagnosis is confirmed by a biopsy and microscopic examination of the tumor tissue. Histologically, DFSP is characterized by a proliferation of spindle-shaped cells with a storiform (whorled) pattern. Immunohistochemistry is often used to aid in diagnosis, with DFSP typically expressing CD34.

The primary treatment for DFSP is surgical excision with wide margins to minimize the risk of recurrence. Mohs surgery, a specialized technique that removes tissue layer by layer while examining each under a microscope, is often recommended for its high cure rates. Other treatment modalities, such as radiation therapy and targeted drug therapies (like imatinib), may be used for unresectable or recurrent cases. Regular follow-up after treatment is important due to the potential for local recurrence.