Primary localized amyloidosis of the urinary bladder: a case series of 31 patients

Mayo Clin Proc. 2000 Dec;75(12):1264-8. doi: 10.4065/75.12.1264.

Abstract

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 chain-related amyloidosis of the urinary bladder. Early eradication with fulguration or laser therapy is indicated. Cystoscopic follow-up is necessary.

MeSH terms

  • Adult
  • Age Distribution
  • Aged
  • Aged, 80 and over
  • Amyloidosis / epidemiology*
  • Amyloidosis / pathology*
  • Amyloidosis / surgery
  • Congo Red
  • Diagnosis, Differential
  • Female
  • Humans
  • Immunohistochemistry
  • Male
  • Middle Aged
  • Minnesota / epidemiology
  • Prognosis
  • Retrospective Studies
  • Sex Distribution
  • Treatment Outcome
  • Urinary Bladder Diseases / epidemiology*
  • Urinary Bladder Diseases / pathology*
  • Urinary Bladder Diseases / surgery
  • Urinary Bladder Neoplasms / diagnosis

Substances

  • Congo Red