膀胱腫瘍を合併したWerner症候群の1例

Abstract

A 41-year-old woman was diagnosed as having Werner's syndrome associated with bladder cancer. The patient noticed sudden, total gross hematuria in September, 1985. Cystoscopy revealed a papillary tumor with a stalk that was accompanied by a daughter tumor and concealed the left ureteral orifice. The tumors were 25 X 20 X 10 mm and 15 X 10 X 5 mm. Double contrast cystograms, computed tomography and transurethral echo showed no invasion of muscle layer. Intravesical instillation of mitomycin (10 mg), cylocide (300 mg) and adriacin (30 mg) was carried out 3 times per week for 4 weeks. Tumor size was reduced, and then TUR was performed. High power section of the removed bladder tumor showed pathologically PNT, TCC, grade II, INF alpha, pTla, lyo and v(-). The patient had such clinical manifestations as short stature with low body weight, thin limbs and stocky trunk, senile face, early graying hair, highpitched voice, bilateral cataracts, osteoporosis, sclerodermia-like signs, flat feet, tendency toward diabetes mellitus and parental consanguinity. Hyaluronic acid was not detected in the urine. To the best of our knowledge, this seems to be the 30th report describing the association of malignancy with Werner's syndrome in Japan; besides, only one other case of bladder cancer in Werner's syndrome has been reported to date in the world.