Clinical features:
(1) painless hematuria which can be severe
(2) variable irrative voiding
(3) variable difficulty voiding with overflow incontinence
(4) evidence of amyloidosis elsewhere if part of systemic disease
(5) variable history of chronic inflammatory disorder
Cystoscopy may show:
(1) one or more mass lesions with or without mucosal ulceration, suspicious for a urothelial neoplasm
(2) rigid bladder wall
(3) yellowish mucosal plaques
A bladder biopsy will show:
(1) There is a deposition of extracellular amorphous hyaline material.
(2) This may be in the lamina propria and/or involving blood vessels.
(3) The material stains positively with Congo red staining with apple green birefringence under polarized light. Immunohistochemistry can also be performed.
(4) There usually is no evidence of malignancy (although concurrent disease is theoretically possible).
