Malignant pigmented villonodular synovitis is an extremely rare and co
ntroversial disease. We describe malignant change in pigmented villono
dular synovitis of the ankle in a patient with an unusually long clini
cal history. Symptoms began at age 21, metastatic disease developed at
age 85, and the patient died 1 year later. The histologic appearance
of the malignant tumor differed from that in most reported cases, in t
hat spindle-shaped cells predominated. Lymph node metastasis also deve
loped, a feature uncommon to soft tissue sarcomas.