This tumor occurs in young adults between 10 and 35 years of age. Male outnumber females 2:1
The tumor may present as a firm,cutaneous or subcutaneous mass, knot or lump.
The xrays show occasional speckled calcification, but may otherwise be unremarkable.
Adequate treatment for this very aggressive and difficult tumor requires early recognition of the true nature of the tumor, followed by complete assessment of the entire extremity and lungs to assess the full extent of the tumor. Surgical treatment requires early radical excision or amputation if the primary tumor is situated in the fingers or toes. Treatment options for tumors which have metastasized are much more limited. Limb salvage may be possible in selected cases.