Arima K, Tsukamoto K, Hori N, Sugimura Y, Tochigi H, Kawamura J, Goto M
Hinyokika Kiyo. 1987 Feb;33(2):269-74.
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.
一名41岁女性被诊断为患有与膀胱癌相关的沃纳综合征。患者于1985年9月突然出现完全性肉眼血尿。膀胱镜检查发现一个带蒂的乳头状肿瘤，伴有一个子肿瘤，遮盖了左输尿管口。肿瘤大小分别为25×20×10毫米和15×10×5毫米。双重对比膀胱造影、计算机断层扫描和经尿道超声检查均显示未侵犯肌层。每周进行3次丝裂霉素（10毫克）、噻替派（300毫克）和阿霉素（30毫克）膀胱内灌注，共4周。肿瘤体积缩小后，进行了经尿道切除术。切除的膀胱肿瘤高倍切片病理显示为乳头状非浸润性膀胱癌，二级，INFα，pTla，ly0和v（-）。患者有以下临床表现：身材矮小、体重低、四肢细、躯干粗壮、老年面容、早生白发、嗓音高亢、双侧白内障、骨质疏松、硬皮病样体征、扁平足、有患糖尿病倾向以及父母近亲结婚。尿液中未检测到透明质酸。据我们所知，这似乎是日本第30例描述恶性肿瘤与沃纳综合征关联的报告；此外，迄今为止世界上仅报道过1例沃纳综合征合并膀胱癌的病例。
