Dermatofibrosarcoma protuberans is a soft tissue sarcoma that is dermal in origin.,The incidence is <0.1% of all malignancies and 1% of soft tissue sarcoma.,Most commonly, it involves trunk (62%) followed by extremities (25%) and head and neck (13%).,It is a slow growing tumor with locally aggressive behavior.,Here, a 50-year-old female diagnosed with orbital dermatofibrosarcoma developed extra-axial component in right frontal region even on chemotherapy.,Hence, the bad prognostic factors are yet to be established in dermatofibrosarcoma protuberans.
Atrophic variant of dermatofibrosarcoma protuberans (DFSP) is a distinct form of DFSP.,Here, we report the case of a 19-year-old woman with a small congenital atrophic plaque on the right precordium.,The lesion remained atrophic for more than 10 years.,Several years earlier, a portion of the plaque became tuberous and enlarged.,Physical examination revealed a 25 × 30 mm erythematous atrophic plaque surrounded by three hard, smooth, and orange-colored nodules of varying sizes on the right precordium, along with visible subcutaneous adipose tissue and cutaneous veins.,Biopsy of the nodule and atrophic plaque revealed dense proliferation of spindle-shaped tumor cells from the dermis to the subcutaneous adipose tissue, and positive immunostaining for CD34 and vimentin in addition to negative staining for factor XIIIa and α-smooth muscle actin.,Reverse transcription polymerase chain reaction (RT-PCR) of the tumor tissue revealed the presence of a COL1A1-PDGFB fusion gene.,Thus, congenital atrophic dermatofibrosarcoma protuberans was diagnosed.,No metastasis to the lungs or regional lymph nodes was found on magnetic resonance imaging.,Wide local excision and split-thickness skin grafting was performed and neither recurrence nor metastasis has been observed for 5 years and 8 months since the surgery.,This case indicates that a congenital atrophic lesion could represent a quiescent phase of DFSP.,Awareness of this rare condition can aid with early diagnosis and thereby improve the prognosis of DFSP.