Abstract
A 77-year-old Japanese woman had noticed multiple brownish pigmented spots with papules on her left upper back since birth. At the same site, she also developed a firm subcutaneous tumor at the age 76. The tumor had rapidly increased in size. Histologically, pigmented spots were naevus spilus, the dermal papule was neurofibroma and the tumor was neurofibrosarcoma. This is the first report of neurofibrosarcoma which derived from segmental neurofibromatosis. No recurrence has since been observed in the 3 years following the operation.
