摘要
目的探讨膀胱淀粉样变性的临床病理特征、诊断及预后。方法回顾性分析1995-11-2016-11解放军总医院住院9例膀胱淀粉样变性患者的临床及病理学资料,进行特殊染色,病理分析。结果9例患者中男性8例,女性1例,平均发病年龄57.8岁(35~71岁)。临床表现为无痛性肉眼血尿。膀胱镜检查结果,7例表现为隆起性病变,2例表现为溃疡性病变;9例均为多发性病变。1例行右肾输尿管全长切除术加膀胱部分切除术,2例行膀胱部分切除术,5例行电灼治疗其中3例加膀胱灌注治疗,1例仅活检而无其他治疗。HE染色,淀粉样变为粉染物,病变累及肌层及血管壁。9例病变刚果红染色均阳性,其中6例高锰酸钾氧化后刚果红染色仍为阳性。随访时间10个月至20年。其中1例随访10个月后因心脏淀粉样变,心肌功能不全去世。其余患者均无复发。结论膀胱淀粉样变临床罕见。临床、影像学及膀胱镜检查均容易误诊为膀胱癌。病变可为局限性或系统性淀粉样变性累及膀胱。局限性淀粉样变性局部切除效果好,系统性淀粉样变性常多次复发,预后差。因经常伴随尿路上皮的病变,因此病理诊断上容易漏诊,需提高认识。
To study the clinicopathologic features,diagnosis,treatment and prognosis of amyloidosis of the urinary tract. Methods Nine cases of amyloidosis of the urinary tract were investigated by clinical findings,histopathology,treatment and prognosis,and related literatures were also reviewed.Results The 9 patients consisted of 8 males and 1 female.The mean age at time of diagnosis was 57.8 years and the ages ranged from 35 to 71 years.All of the patients presented with gross painless haematuria.Cystoscopy revealed multiple lesions with protuberant lesion ( 7 cases) or ulcerative lesion ( 2 cases).The treatment consisted of right kidney ureter complete resection plus partial cystectomy ( 1 case ),partial cystectomy ( 2 cases),electrocautery ( 5 cases) plus intravesical instillation ( 3 cases),and biopsy only without any other treatment ( 1 case).On hematoxylin-eosin stain,the amyloid appeared as eosinophilic amorphous deposits,involving the muscular layer and vessel wall.Congo red staining was positive in 9 cases,6 of which were positive for Congo red staining after potassium permanganate.The follow-up period ranged from 10 months to 20 years.One patient was died of cardiac amyloidosis and myocardial dysfunction after 10 months of diagnosis.No recurrence was found in the rest of the patients. Conclusions Amyloidosis of the urinary tract is a very rare disease that mimics neoplasia clinically,radiologically and cystoscopically,which can be focal or systemic amyloidosis involving the urinary tract.Localized amyloidosis has a good prognosis after local excision.However,systemic amyloidosis often recurred many times with poor prognosis.The lesion is easily missed in pathological diagnosis as the accompanying of the urinary tract epithelium change,which needs a deeper understanding.
作者
余春开
宋志刚
YU Chun-kai;SONG Zhi-gang(Department of Pathology,Beijing Shijitan Hospital,Capital Medical University,Beijing 100038;Department of Pathology,the PLA General Hospital,Beijing 100853,China)
出处
《诊断病理学杂志》
2019年第8期499-502,共4页
Chinese Journal of Diagnostic Pathology
关键词
尿路系统
膀胱
淀粉样变性
诊断
预后
urinary tract
urinary bladder
amyloidosis
diagnosis
prognosis
