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Eble JN, Sauter G., Epstein JI, Sesterhenn IA - iarc

Eble JN, Sauter G., Epstein JI, Sesterhenn IA - iarc

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Lymphomas<br />

A. Marx<br />

Definition<br />

Malignant lymphoma is a malignant lymphoid<br />

neoplasm which may occur in the<br />

urinary bladder as a primary or part of a<br />

systemic disease.<br />

Epidemiology<br />

Lymphomas constitute about 5% of nonurothelial<br />

tumours of the urinary tract.<br />

More than 90% affect the bladder {1730},<br />

constituting less than 1% of bladder neoplasms<br />

{86,106,530}. Secondary lymphoma<br />

of the bladder is common (12-<br />

20%) in advanced stage systemic lymphoma,<br />

shows a slight male predominance<br />

and may occur in children {885,<br />

1297}. Primary lymphomas of the bladder<br />

{1297,1946,2793} and urethra {127,<br />

398,1040,1414} are rare, affect mainly<br />

females (65-85%) and occur at an age of<br />

12 - 85 (median 60) years. In one series<br />

only 20% of cases were primary lymphomas<br />

{1297}.<br />

Etiology<br />

The etiology of urinary tract lymphomas is<br />

unclear. Chronic cystitis is regularly<br />

encountered in MALT lymphoma of the<br />

bladder {1297,1402,2034}, but less frequently<br />

(20%) in other lymphomas {1946}.<br />

EBV and HIV infection have been reported<br />

in rare high grade urinary tract lymphoma<br />

(UTL) {1257,1692,1947}. Schistosomiasis<br />

was associated with a T-cell lymphoma of<br />

the bladder {1820}. Posttransplant lymphoproliferative<br />

disease restricted to the<br />

ureter allograft may occur after renal<br />

transplantation {591,2360}.<br />

Clinical features<br />

The most frequent symptom of urinary<br />

tract lymphomas is gross hematuria, followed<br />

by dysuria, urinary frequency, nocturia<br />

and abdominal or back pain<br />

{1297,1946}. Fever, night sweats, and<br />

weight loss or ureteral obstruction with<br />

hydronephrosis and renal failure occur<br />

almost only in patients with secondary<br />

urinary tract lymphomas due to retroperitoneal<br />

disease. Antecedent or concurrent<br />

MALT lymphomas in the orbit {1297}<br />

and stomach {1396}, and papillary<br />

urothelial tumours rarely occur {2034}.<br />

Urinary tract lymphomas affect the renal<br />

pelvis, ureter, bladder and urethra.<br />

Primary urinary tract lymphomas are confined<br />

to the urinary tract, while secondary<br />

lymphoma results from disseminated<br />

lymphoma/leukaemia. Secondary bladder<br />

lymphoma as the first sign of disseminated<br />

disease is termed "nonlocalized<br />

lymphoma" with a much better prognosis<br />

than "secondary [recurrent] lymphoma"<br />

in patients with a history of lymphoma<br />

{1297}.<br />

Macroscopy<br />

Bladder lympomas may form solitary<br />

(70%) or multiple (20%) masses or diffuse<br />

thickening (10%) of the bladder<br />

wall. Ulceration is rare (

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