Elsevier

Mayo Clinic Proceedings

Volume 75, Issue 12, December 2000, Pages 1264-1268
Mayo Clinic Proceedings

Original Article
Primary Localized Amyloidosis of the Urinary Bladder: A Case Series of 31 Patients

https://doi.org/10.4065/75.12.1264Get rights and content

Objective

To evaluate the presentation and prognosis of primary localized amyloidosis of the urinary bladder.

Patients and Methods

The medical records of 31 patients with primary localized amyloidosis of the urinary bladder were reviewed. Immunohistochemical amyloid typing was performed on bladder biopsy specimens from 27 patients.

Results

The median age of the 22 men and 9 women was 55 years. Twenty-four patients (77%) presented with gross hematuria (associated with irritative urinary tract symptoms in 6 patients), and 7 (23%) had only irritative lower urinary tract symptoms. Multiple bladder areas were involved in 20 patients (65%), a single area was involved in 8 (26%), and diffuse involvement was present in 3 (10%). Twenty-four patients had immunoglobulin light chain, and 3 had transthyretin-related amyloid. Local recurrences were common. None of the patients developed systemic amyloidosis.

Conclusion

Primary localized amyloidosis of the urinary bladder can be easily confused with a neoplasm. Immunohistochemical amyloid typing is important. Transthyretin-related amyloid of the bladder requires no further work-up. Repeated work-ups for systemic amyloidosis are unnecessary for patients with light chainrelated amyloidosis of the urinary bladder. Early eradication with fulguration or laser therapy is indicated. Cystoscopic follow-up is necessary.

Section snippets

PATIENTS AND METHODS

We identified 31 patients with primary localized amyloidosis of the bladder who were seen at the Mayo Clinic in Rochester, Minn, between 1944 and 1996. Patients with primary systemic amyloidosis and amyloidosis secondary to rheumatic conditions, infections, and malignancies were excluded. Eight of the patients have been described previously by authors from our institution'; this article includes these 8 patients and their follow-up information as well as 23 patients examined subsequently at the

Sex and Age Distribution

The 31 patients consisted of 22 men (71%) and 9 women (29%). The median age of the 31 patients at diagnosis was 55 years (range, 28-80 years). In men, the disease was almost evenly distributed over the fifth, sixth, and seventh decades of life, whereas in most women (56%), the disease was diagnosed in the sixth decade of life (Table 1).

Symptoms

Of the 31 patients, 24 (77%) presented with gross, painless hematuria. In 6 of these patients, the hematuria was associated with irritative symptoms of the lower

DISCUSSION

Amyloidosis is characterized by deposition, in various tissues, of a homogeneous eosinophilic material that has a nonbranching branching fibrillar ultrastructure. Most cases occur in a generalized form as a manifestation of an underlying plasma cell neoplasm (myeloma) or plasmacytic dyscrasia. Systemic amyloidosis is a progressive and usually fatal disease. The amyloid fibrils in primary systemic amyloidosis are derived from monoclonal immunoglobulin light chains (AL type). Localized

CONCLUSION

Primary localized amyloidosis of the urinary bladder is a rare disorder. It is of interest to the generalist and the urologist because it can be easily confused with carcinoma. Immunohistochemical subtype determination is helpful because transthyretin amyloid of the bladder has a presentation similar to that of the AL type. The finding of transthyretin amyloid requires no further evaluation. Repeated evaluations for systemic amyloid are unnecessary for patients with the AL subtype in that none

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Cited by (115)

  • Primary localised bladder amyloidosis – A case report with review of the literature

    2022, Urology Case Reports
    Citation Excerpt :

    A high degree of variability was noted in both follow-up duration following initial treatment and rate of disease recurrence. Zhou et al. (2014) reported a median follow-up period of 64 months (IQR 33.5–90.5 months) with recurrence rates as low as 0%, whilst Tirzaman et al. (2000) reported a median follow-up period of 120 months (IQR 24–864 months) and the rate of recurrence was as high as 42%.2,5 Our review of the literature thus far has shown that recurrences are relatively common in localised bladder amyloidosis, whilst progression to systemic amyloidosis has not been reported.

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Dr Tirzaman is now with the University of Massachusetts Medical School of Worcester.

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