The tumor has two subtypes - proximal and distal. The distal subtype is most prevalent in adolescents and young adults between 10 and 35 years of age. Male patients outnumber females by about 2:1.
The tumor nearly always presents as a slow growing mass. There may be minimal or no pain and no loss of function.
The xrays show occasional speckled calcification, but may otherwise be unremarkable. Plain xrays are recommended to rule out other types of lesions and evaluate any possible bone involvement.
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.