A Case of Primary Lymphoma of the Bladder Managed With Multimodal Therapy

Kaya Horasanli; * Mustafa Kadihasanoglu; Oznur Tas Aksakal; Aysim Ozagari and Cengiz Miroglu

Disclosures

Nat Clin Pract Urol. 2008;5(3):167-70. 

In This Article

DiscussIon of Diagnosis

Although the bladder is secondarily involved in 10–20% of terminal non-Hodgkin's lymphoma cases,[1] primary lymphomas of the bladder are very uncommon and represent less than 1.0% of vesical tumors and less than 0.2% of extranodal lymphomas.[2,3] The most common sites of primary extranodal malignant lymphoma are the stomach, connective tissues and skin. Primary lymphoma of the bladder was first described by Eve in 1885.[4] Approximately 100 cases have been reported in the literature. The etiology of primary lymphoma of the bladder has not been elucidated, partly because of the rarity of the condition. According to one theory, lymphoma follows chronic cystitis and an associated increase in the amount of lymphoid tissue found in the lamina propria.[2,5,6,7] As a number of cases have been reported in the literature in the absence of chronic cystitis, we postulate that the tumor might originate in the lymphoid tissue derived from the embryonic cloaca.[6] Among primary terminal lymphomas, low-grade lymphomas—including the subtypes of mucosa-associated lymphoid tissue (MALT)—are common.[8,9] Primary lymphoma of the bladder affects 6.5 times more women than men, and the tumor typically appears in patients between the ages of 20 and 85 years (at a median of 64 years).[10]

The major symptoms of primary lymphoma of the bladder are intermittent hematuria, urinary frequency and dysuria. This disease presents as a solitary tumoral mass. A history of chronic cystitis has been shown to be a preceding feature in some cases of primary bladder lymphoma;[10,11] approximately 20% of patients present with such a history.[10,12] The current patient did not have a history of recurrent urinary infection. Hydronephrosis is an uncommon feature of the tumor. The tumor does not typically involve the ureteral orifices, and involvement of the entire bladder wall is also a rare phenomenon.[13,14] The current patient's tumor involved the right ureteral orifice and caused hydronephrosis of the right kidney; this is a very uncommon presentation of primary lymphoma of the bladder.

At presentation, the patient was hemodynamically stable; there were no signs of clinical anemia, and findings on abdominal examination were normal. Microscopic analysis of her urine showed the presence of erythrocytes but not lymphocytes. No evidence of malignant cells was found on cytologic assessment. Serum levels of urea, creatinine and electrolytes were measured and were found to be normal, indicating that the patient's hydronephrosis had not resulted in impaired renal function. CT of the upper abdomen and chest showed no evidence of systemic disease. There was also no evidence of lymphadenopathy or hepatosplenomegaly on CT. Bone marrow aspirate and a bone biopsy sample showed no sign of lymphoma infiltration.

This patient had hematuria, dysuria, urinary frequency and urgency. Other possible causes of these symptoms are cystitis, interstitial cystitis, bladder carcinoma and urinary stones. Ultrasonography and CT of the abdomen showed no sign of urinary stones. No growth of organisms was observed on midstream urine culture, ruling out cystitis. Cystoscopy showed an irregular lobular, solid and ulcerative mass arising from the right wall of the bladder.

Histopathological and immunohistochemical studies are absolutely necessary for the diagnosis of bladder lymphoma, as the clinical and radiological features of this tumor are similar to those of urothelial carcinomas of the bladder. The most common macroscopic findings are solitary masses located in the lateral walls of the bladder.[15] The most common primary malignant lymphomas of the bladder are low-grade B-cell-derived non-Hodgkin's lymphomas of the MALT type, including Burkitt's and plasmacytoid lymphomas,[3] which are associated with good prognoses. Approximately 20% of primary bladder lymphomas are high-grade neoplasms.[10] High-grade primary bladder lymphomas are mostly of the diffuse large-B-cell type.[1,3,16] In this patient, strong positive staining for LCA and CD20 (Figure 2B) indicated a B-cell-derived lymphoma, while immunohistochemical analyses negative for cytokeratin, cytokeratin 7 and CD45RO ruled out the possibility of carcinoma.

Primary malignant lymphoma of the bladder usually exhibits a silent course with a favorable prognosis. The prognosis is associated with the tumor grade and stage at presentation. If a high-grade lymphoma of the bladder is diagnosed by pathological examination, systemic lymphoma should be excluded via clinical and radiographic examinations, including CT of the abdomen and chest. Also, bone marrow aspirate and bone biopsy samples should be analyzed to exclude the possibility of systemic lymphoma. In the current patient, clinical and radiographic examinations excluded systemic lymphoma and confirmed a focused lymphoma of the bladder.

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