Clinical Features:
- Benign tumor presenting in the first two years of life
- Male predominance
- Solitary dermal and subcutaneous mass, most commonly on the shoulder, proximal upper limb, groin, back or forearm
- Presents as a firm, mobile sesion up to 10 cm in diameter
- May rarely recur if incompletely excised
Histologic Features:
- Ill-defined lesion mainly within the deep dermis and subcutis, composed of varying amounts of:
- Disordered fibrous tissue: coarse collagen bundles with blood vessels and inflammation
- Fascicles of myofibroblasts
- Mature adipose tissue
- Foci of myxoid tissue containing primitive mesenchymal cells with a whorled configuration