Imaging findings of head and neck dermatofibrosarcoma protuberans

Dermatofibrosarcoma protuberans is a rare, locally aggressive sarcoma of the skin in children and adults, usually involving the trunk and extremities and less commonly the head and neck. Despite clinical reports in the literature on the management of dermatofibrosarcoma protuberans, there are limited articles describing its imaging features.

We retrospectively reviewed the demographics and imaging findings in all 24 patients with pathologically proven dermatofibrosarcoma protuberans of the head and neck seen at a tertiary cancer center between 2001 and 2010.

Twenty-two of the 24 lesions were nodular and well circumscribed; 19 of the 24 were located on the scalp. On imaging, all 24 lesions involved subcutaneous tissues. The lesions ranged in size from 0.6-9.5 cm (mean, 3.7 cm; standard deviation, 2.3 cm). Twelve lesions involved the soft tissues either at or extending directly to the midline. Thirteen lesions were associated with bulging of the skin surface. Fourteen lesions were imaged with CT and 14 with MR imaging. Whereas variable enhancement patterns were noted on CT and MR imaging, dermatofibrosarcoma protuberans was usually T2-hyperintense and demonstrated marked enhancement. None of the lesions was associated with bone invasion, perineural spread, or nodal/distant metastasis.

Knowledge of the imaging characteristics of dermatofibrosarcoma protuberans may alert neuroradiologists to include dermatofibrosarcoma protuberans in the differential diagnosis of lesions about the head and neck with similar imaging characteristics.