Most patients are young adults, with 75% percent of cases occurring in persons under 40, but children and the elderly may be affected.
The tumor may present as a small, firm superficial or deep nodule or a focal cluster of nodules. This tumor is frequently misdiagnosed as a skin condition, warts, or corns, and a correct diagnosis may be delayed with serious medical and legal consequences.
Contrast-enhanced MRI and PET CT imaging of the entire limb is essential to detect the complete extent of the disease and locate the frequent local and regional metastases.
For surgically controllable disease, surgical removal with a wide margin is mandatory.