Primary Low-Grade B-Cell Lymphoma of Mucosa-Associated Lymphoid Tissue Type Arising in the Urinary Bladder

Abstract

Context.—Primary lymphoma of the urinary bladder is rare. Only 84 cases have been reported in the English literature to date, and none of these cases has had molecular confirmation of clonal immunoglobulin gene rearrangement. Objectives.—To review all cases with primary urinary bladder lymphoma in our records, to classify them using the REAL classification, to confirm their immunophenotype and genotype, and to determine their outcome. Design.—We identified 4 cases of primary urinary bladder lymphoma in our medical records from a 30-year period. Immunohistochemical detection of immunoglobulin light chains and molecular analysis of immunoglobulin heavy-chain genes using the polymerase chain reaction were performed on paraffin-embedded material. Results.—All patients were older than 60 years. The male-female ratio was 1:3. All patients had a history of chronic cystitis. Histologic features of mucosa-associated lymphoid tissue lymphoma with centrocyte-like cells, plasmacytoid B cells, or both were observed in all cases. Monoclonality of B cells was demonstrated by immunohistochemistry, polymerase chain reaction, or both methods in every case. All patients presented with stage IAE disease, were treated with radiotherapy alone, and have been in continuous complete remission for 2 to 13 years. Conclusions.—Primary bladder lymphomas are usually of low-grade mucosa-associated lymphoid tissue type. They are more common in females and are associated with a history of chronic cystitis. Lymphoepithelial lesions are seen only in association with areas of cystitis glandularis. B-cell clonality is readily demonstrable by immunohistochemistry and/or polymerase chain reaction analysis. Local radiotherapy appears to confer long-term control.

Bladder mucosa-associated lymphoid tissue lymphoma progressed from chronic cystitis along with a comparative genetic analysis during long-term follow-up: a case report

“…Bladder MALT lymphoma may originate from underlying prolonged chronic inflammation of the bladder (4-6), though precise mechanisms have not been clarified, unlike Helicobacter pylori infection in gastric MALT (12,13). Lymphoepithelial lesion (LEL) has been reported one of characteristic findings of MALT lymphoma in general, and can exist in areas of cystitis glandularis of the bladder without lymphoid tissue (14)(15)(16). LEL is rare in patients with bladder MALT lymphoma, and its identification is not essential for diagnosis.…”

“…Few previous reports have focused on the genetic analysis of bladder lymphoma; we identified only six cases in three reports within the scope of our investigation. Among these cases, a monoclonal gene rearrangement of IGH was identified by PCR in five patients (16,19,20). FISH was performed in only one patient, and gene translocation of IGH-MALT1 was confirmed (20).…”

“…The severe perineal pain was transiently aggravated by radiation cystitis as expected, and the PUF score peaked immediately after completing the treatment; however, it then dramatically decreased to below the pretreatment score. Although the standard treatment for bladder lymphoma has not been established, RT after TUR-BT is often performed because it is expected to result in a favorable prognosis (6,16,21). Severe perineal pain is often transiently aggravated by radiation cystitis.…”

Development and Validation of Survival Nomograms in Patients with Primary Bladder Lymphoma

“…PBL tends to develop in middle-aged females, with a reported male-to-female ratio of 1:3 by Al-Maghrabi et al to 1:6.5 by Ohsawa et al, and most patients are diagnosed in their sixties [ 8 , 9 , 10 ]. The most common and obvious clinical symptom of PBL is gross hematuria, and symptoms such as urinary frequency, dysuria, abdominal pain, and weight loss may also occur [ 8 , 11 , 12 ].…”

“…Most PBL are low-grade tumors, while high-grade tumors only account for 20% [ 2 ]. The most common type among low-grade PBL is extranodal marginal zone/mucosa-associated lymphoid tissue (MALT) lymphoma, accounting for 86% of low-grade cases and 53% of all cases [ 5 , 10 , 13 ], and the most common type among high-grade PBL is diffuse large B-cell lymphoma (DLBCL) [ 12 ]. Currently, there are no standardized protocols or guidelines for the treatment of PBL.…”