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 local
ized 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 lowe
r urinary tract symptoms. Multiple bladder areas were involved in 20 patien
ts (65%), a single area was involved in 8 (26%), and diffuse involvement wa
s 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 eas
ily confused with a neoplasm, Immunohistochemical amyloid typing is importa
nt. Transthyretin-related amyloid of the bladder requires no further work-u
p, Repeated work-ups for systemic amyloidosis are unnecessary for patients
with light chain-related amyloidosis of the urinary bladder. Early eradicat
ion with fulguration or laser therapy is indicated. Cystoscopic follow-up i
s necessary.