융기성 피부섬유육종의 치험2례 = DERMATOFIBROSARCOMA PROTUBERANS -TWO CASES REPORT-

다국어 초록 (Multilingual Abstract)

Dermatofibrosarcoma protuberans is an uncommon soft tissue neoplasm that usually presents as a painless, often long standing mass arising in the dermis of the skin.
In most patients, the nodule enlarges slowly and painlessly for a period of 3 months to 2 years as it infiltrates the adjacent tissue and becomes multinodular.
At the later stage, however, its growth rate accelerates, and it can ulcerate, bleed, or become painful. Histologically, on light microscopy, this tumor shows the characteristic cartwheel patterns, with the fibroblasts arranged radially about a small central hub of fibrous tissue, and on electron microscopy, fairly monotonous population of spindle-shaped cells with a few stellate cells. Many of the spindle cells contained intracytoplasmic aggregates of microfilaments with dense bodies and were partially surrounded by basal lamina, interpreted as being typical of myofibroblasts. Other cells showed typical features of fibroblasts
Dermatofibrosarcoma protuberans typically arises on the trunk and proximal extremities. head and neck and scalp lesions have also been described.
It is more frequent in men with a peak incidence during the third decade of life.
We have encountered 2 cases, develpoed on the both ear lobules of a 25-year-old woman and the right lower of a 7-year-old child ; but there were no evidence of metastasis.
A scapular fasciocutaneous free flap and a skin graft covered large defects resulting from wide tumor excision.
Follow-up at 1 year showed the patient doing well with no evidence of local or regioal recurrence.