Review Question - QID 7497

Dermatofibrosarcoma is a rare, monoclonal, cutaneous sarcoma arising in the dermis in the trunk, 47%; lower extremity, 20%; upper extremity, 18%; and head and neck, 14%. Symptoms may be present for a duration of 6 months to 30 years. It may occur at any age, with a peak incidence in the fourth decade; it is more common in men than women (3:2). Most tumors are superficial and less than 5 cm, but 3% are larger than 10 cm. They present early as pink or violet-red plaques surrounded by telangiectatic skin. A nodular growth pattern with ulceration and attachment to deeper structures is observed in advanced and/or recurrent cases. Most cases (85% to 90%) are low grade but (5% to 15%) contain focal, high-grade fibrosarcomatous areas with intermediate-grade tumor (DFSP-FS). MRI is useful in ascertaining tumor extent and depth of invasion. Tumor cells exhibit a storiform growth pattern and infiltrate adjacent adnexal structures and adipose tissue. Fibrosarcomatous transformation can occur over time and is recognized by increased mitotic activity. Because some tumors express platelet derived growth factor, they may be responsive to imatinib. Ewing's sarcoma, extraskeletal myxoid chondrosarcoma, liposarcoma, and synovial sarcoma are generally deep-seated tumors that can but very rarely cause skin ulceration.