At age 25 a Japanese woman noticed an elastic-hard nodule 2 cm in diam
eter on the anterior side of her right leg. The nodule had developed a
n ulcer in its center. Simple resection was performed several times. H
owever, the lesion recurred repeatedly. The patient underwent amputati
on of the right leg at the age of 34, because the diagnosis of epithel
ioid sarcoma was established histologically. No recurrence was observe
d for 9 years. Recently, the patient noticed multiple painful, ulcerat
ive nodules about 1 cm in diameter on her scalp, trunk, and extremitie
s. She refused extensive resection for a religious reason and died of
massive hematemesis. Autopsy revealed metastatic epithelioid sarcoma i
n the skin, lungs, kidneys, pancreas, transverse colon, thyroid, and s
ternum. Chromosomal analysis of the tumor revealed various aberrations
and an N-ras oncogene mutation.