Huang Chao-Yuan, Shun Chia-Tung, Huang Kuo-How, Chen Jun, Pu Yeong-Shiau
Department of Urology, National Taiwan University Hospital, Taipei, Taiwan, ROC.
J Formos Med Assoc. 2006 Feb;105(2):164-7. doi: 10.1016/S0929-6646(09)60339-9.
Amyloidosis is a systemic disease that usually occurs in the gastrointestinal tract or in muscular or adipose tissue. Primary amyloidosis of the urinary bladder is a rare disease that can mimic bladder cancer on cystoscopic examination as well as in its clinical presentation of painless gross hematuria. This report describes a 49-year-old male with repeated painless gross hematuria, who underwent transurethral resection of a suspected bladder tumor. Pathologic examination revealed papillary urothelial hyperplasia with vascular ectasia and no signs of malignancy. Massive gross hematuria occurred 2.5 years later. Cystoscopy showed multiple papillary lesions with yellowish-brown submucosal plaques on the posterior bladder wall. A second transurethral tumor resection was performed and histologic examination revealed plasma cell infiltration and eosinophilic amorphous deposits in the subepithelial stroma and vascular wall. The deposits were positive for Congo red and apple-green birefringence under polarized light examination but negative for Masson's trichrome stain, indicating that they were not fibrotic in nature. Hence, the diagnosis of amyloidosis of the urinary bladder was confirmed. Screening for amyloidosis was negative in other organ systems and the patient has remained disease-free up to the last follow-up 4 years after the second transurethral resection. Amyloidosis should be considered in the differential diagnosis of patients with recurrent hematuria who have symptoms characteristic of bladder cancer but negative pathologic study for malignancy. Correct diagnosis relies on clinical alertness and the use of a special staining technique during pathologic examination.
淀粉样变性是一种全身性疾病,通常发生在胃肠道、肌肉或脂肪组织中。膀胱原发性淀粉样变性是一种罕见疾病,在膀胱镜检查及临床表现为无痛性肉眼血尿时,可能会被误诊为膀胱癌。本报告描述了一名49岁男性,反复出现无痛性肉眼血尿,接受了经尿道疑似膀胱肿瘤切除术。病理检查显示为伴有血管扩张的乳头状尿路上皮增生,无恶性迹象。2.5年后出现大量肉眼血尿。膀胱镜检查显示膀胱后壁有多个乳头状病变及黄褐色黏膜下斑块。进行了第二次经尿道肿瘤切除术,组织学检查显示上皮下基质和血管壁有浆细胞浸润及嗜酸性无定形沉积物。这些沉积物刚果红染色阳性,偏振光检查呈苹果绿双折射,但Masson三色染色阴性,表明它们本质上不是纤维化的。因此,确诊为膀胱淀粉样变性。其他器官系统的淀粉样变性筛查为阴性,该患者在第二次经尿道切除术后4年的最后一次随访时仍无疾病复发。对于有膀胱癌特征性症状但恶性病理研究阴性的复发性血尿患者,鉴别诊断时应考虑淀粉样变性。正确诊断依赖于临床警觉性及病理检查时使用特殊染色技术。