Malignant peripheral nerve sheath tumor (malignant schwannoma) of urinary bladder in von Recklinghausen neurofibromatosis.

Von Recklinghausen neurofibromatosis occurs in 1 in 3,000 live births; however, urologic manifestations are rare. There have been over 40 reported cases of neurofibroma of the bladder. Malignant degeneration of subcutaneous neurofibroma occurs in about 5 to 10 percent of patients. We believe we present the second reported case of a malignant peripheral nerve sheath tumor involving the bladder in a patient with this disorder.