Myxoid Dermatofibrosarcoma Protuberans of the Vulva with Myoid Nodules: Clinicopathologic and Immunohistochemical Study of a Case

Dermatofibrosarcoma protuberans is a slow growing dermal spindle cell tumor seldom seen in the vulva and its myxoid variant, a rare type of dermatofibrosarcoma protuberans is characterised by extensive myxoid degeneration. We present the case of a 62 year old woman with an enlarging vulval swelling. Mass was excised surgically. Histopathologically the tumor consisted of uniform spindle-shaped cells showing strong positivity with CD34. In addition to the typical storiform pattern and lace like infiltration, prominent myxoid stromal changes were seen. Herein we report an interesting case of myxoid dermatofibrosarcoma protuberans, uncommonly reported in the dermatopathology literature.