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Disorders of the ureter are uncommon and include congenital disorders, neoplasms, and reactive conditions. A few merit brief mention.
Ureteropelvic junction (UPJ) obstruction, a congenital disorder, results in hydronephrosis. It usually manifests in infancy or childhood and is much more common in boys than in girls. It is the most frequent cause of hydronephrosis in infants and children.
Malignant tumors of the ureter are pathologically similar to those arising in the renal pelvis, calyces, and bladder (discussed later). Most are urothelial carcinomas.
Retroperitoneal fibrosis is an uncommon cause of ureteral narrowing or obstruction characterized by a fibrous proliferative inflammatory process encasing the retro-peritoneal structures and causing hydronephrosis. The disorder occurs in middle to old age. At least a proportion of these cases occur in association with IgG4-related disease, characterized by fibroinflammatory lesions rich in IgG4-secreting plasma cells (Chapter 5). Other cases are associated with drug exposures (ergot derivatives, adrenergic blockers), radiation, infection, prior surgery, or malignant disease (lymphomas, urinary tract carcinomas). Most cases, however, have no obvious cause and are considered primary, or idiopathic (Ormond disease).
A bladder or vesical diverticulum consists of a pouchlike evagination of the bladder wall. Diverticula may be congenital but more commonly are acquired lesions that arise as a consequence of persistent urethral obstruction caused, for example, by benign prostatic hyperplasia. Although most diverticula are small and asymptomatic, they sometimes lead to urinary stasis predisposing to recurrent urinary tract infections and bladder stone formation.
Cystitis takes many forms.
Bacterial cystitis is common, particularly in women. The most common etiologic agents are coliform bacteria.
Hemorrhagic cystitis may occur in patients receiving cytotoxic anti-tumor drugs, such as cyclophosphamide, and sometimes complicates adenovirus infection.
Interstitial cystitis causes a chronic pelvic pain syndrome, typically in women. It is characterized by suprapubic pain that increases with bladder filling and is relieved by bladder emptying, leading to very frequent urination during both day and night. Other symptoms include urgency, hematuria, and dysuria. Cystoscopic findings are nonspecific and include petechial hemorrhages. Up to 50% of patients have spontaneous remissions. Late in the course, transmural fibrosis may ensue, leading to a contracted bladder.
Malakoplakia is an uncommon inflammatory disease that most commonly occurs in the bladder. It results from defects in the phagocytic or degradative function of macrophages. As a result of this defect, undigested bacterial products accumulate within distended phagosomes, which are seen in histologic sections as abundant granular material within the cytoplasm of macrophages. The abnormal macrophages also contain laminated mineralized concretions known as Michaelis-Gutmann bodies, which result from deposition of calcium salts in the enlarged lysosomes.
Polypoid cystitis is an inflammatory condition resulting from irritation to the bladder mucosa in which the urothelium is thrown into broad bulbous polypoid projections as a result of marked submucosal edema. Polypoid cystitis may be confused with papillary urothelial carcinoma both clinically and histologically.