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CASE REPORT
Year : 2022  |  Volume : 18  |  Issue : 6  |  Page : 1814-1816

Primary urinary bladder marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue


Department of Radiology, The University of Tokyo Hospital, Tokyo, Japan

Date of Submission09-Feb-2021
Date of Acceptance21-Jan-2022
Date of Web Publication15-Jul-2022

Correspondence Address:
Atsuto Katano
7-3-1 Hongo, Bunkyo-ku, Tokyo, 113-8655
Japan
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jcrt.jcrt_238_21

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 > Abstract 


Primary urinary bladder marginal zone B-cell lymphoma of the mucosa-associated lymphoid tissue (MALT) is an extremely rare disease. Here, we have reported a case of MALT lymphoma that was successfully treated with transurethral resection of the bladder tumor (TUR-BT) and radiotherapy. A 65-year-old woman presented with macroscopic hematuria. She had a history of chronic cystitis. Cystoscopy of the bladder revealed a submucosal tumor measuring 4 cm in the trigone of the bladder floor. Magnetic resonance imaging showed that the lesion had intermediate intensity on T2-weighted images. TUR-BT was performed, and the lesion was diagnosed with marginal zone B-cell lymphoma of MALT histopathologically. Positron emission tomography (PET) showed a slightly higher fluorine-18-deoxyglucose (FDG) accumulation, with a maximum standardized uptake value of 17.3, than the physiological accumulation in the tumor resection area of the bladder, with no obvious abnormal accumulation outside the bladder. The patient underwent field radiotherapy at a dose of 30 Gy in 15 fractions, administered in 5 fractions per week. She developed grade 2 cystitis as an acute radiation-related adverse event, without any hematological adverse events. On PET at 5 months after radiotherapy, FDG accumulation in the posterior wall of the bladder was obscured and remained regressed after 2 years.

Keywords: Mucosa-associated lymphoid tissue lymphoma, radiotherapy, urinary bladder


How to cite this article:
Katano A, Yamashita H. Primary urinary bladder marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue. J Can Res Ther 2022;18:1814-6

How to cite this URL:
Katano A, Yamashita H. Primary urinary bladder marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue. J Can Res Ther [serial online] 2022 [cited 2022 Dec 3];18:1814-6. Available from: https://www.cancerjournal.net/text.asp?2022/18/6/0/351104




 > Introduction Top


Primary urinary bladder marginal zone B-cell lymphoma of the mucosa-associated lymphoid tissue (MALT) is an extremely rare disease. MALT lymphoma is classified as low-grade non-Hodgkin lymphoma and is clinically divided into gastric and nongastric MALT lymphomas. Nongastric MALT lymphomas occur in the salivary glands, skin, thyroid, mammary glands, and conjunctiva. However, they rarely occur in the bladder.[1]

Chronic inflammation has been suggested to be the underlying cause that leads to the development of MALT lymphoma. Furthermore, Helicobacter pylori infection in the stomach and Hashimoto's disease in the thyroid gland have been shown to be associated with the development of MALT lymphoma.[2] In the bladder, MALT is caused by chronic inflammation such as cystitis.[3] Some chromosomal translocations are associated with the pathogenesis of MALT lymphomas, including t(14;18)(q32;q21), t(11;18)(q21;q21), t(1;14)(p22;q32), and t(3;14)(p14.1;q32).[4] In particular, t(11;18)(q21;q21), which is a fusion gene between the apoptosis inhibitor protein 2 on chromosome 11 (q21) and the MALT lymphoma-associated translocation 1 on chromosome 18 (q21), is the most commonly found translocation in MALT lymphomas. This chromosomal abnormality is clinically associated with resistance to H. pylori eradication therapy and advanced-stage MALT lymphoma.[5]

Primary lymphoma of the bladder is a rare malignancy, and there is limited literature to guide its treatment. Here, we have reported a case of primary urinary bladder MALT lymphoma that was successfully treated with transurethral resection of the bladder tumor (TUR-BT) and radiotherapy.


 > Case report Top


A 65-year-old woman presented with macroscopic hematuria and a history of chronic cystitis. Cystoscopy of the bladder revealed a submucosal tumor measuring 4 cm in the trigone of the bladder floor. Magnetic resonance imaging showed that the lesion located at the posterior wall of the bladder had intermediate intensity on T2-weighted images [Figure 1]. There was no significant increase in serum lactate dehydrogenase (203 U/mL) and soluble interleukin-2 receptors (176 U/mL) levels. The urine cytology results showed mild atypia, classified as class II. Considering these investigations, TUR-BT was performed. Histopathological examination of the lesion revealed the presence of follicular colonization, and most of the infiltrating atypical lymphocytes were immunohistochemically positive for CD20(+), CD79a(+), and BCL2(+) and negative for CD3(-), AE1/AE3(-), CD5(-), CD10(-), CD23(-), cyclin D1(-), terminal deoxynucleotidyl transferase(-), BCL6(-), and EBER-ISH(-). The Ki-67 labeling index was estimated to be approximate 10%–20%. Immunoglobulin light-chain restriction was not detected on in situ hybridization for kappa and lambda light chains.
Figure 1: Magnetic resonance imaging of the pelvis demonstrating intermediate intensity in the tumor on sagittal T2-weighted images

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After TUR-BT, positron emission tomography (PET) combined with computed tomography (CT) showed a slightly higher fluorine-18-deoxyglucose (FDG) accumulation, with a maximum standardized uptake value of 17.3 than the physiological accumulation in the tumor resection area of the bladder. Hence, residual MALT lymphoma lesions were suspected [Figure 2]a. PET-CT showed no obvious abnormal accumulation outside the bladder. Serum anti-H. pylori immunoglobulin G antibody test results were negative. Bone marrow biopsy results were also negative. Then, the patient was diagnosed with stage IE MALT lymphoma of the bladder according to the Ann Arbor classification.
Figure 2: (a) Positron emission tomography combined with computed tomography showing a high fluorine-18-deoxyglucose accumulation, with a maximum standardized uptake value of 17.3, on the posterior wall of the bladder. (b) Obscuring of the accumulation at 5 months after radiotherapy

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She received involved-field radiotherapy at a dose of 30 Gy in 15 fractions, administered in 5 fractions per week [Figure 3]. She developed grade 2 cystitis as an acute radiation-related adverse event. On PET-CT at 5 months after radiotherapy, FDG accumulation in the posterior wall of the bladder was obscured [Figure 2]b and remained regressed for 2 years.
Figure 3: The green and red lines show the clinical target volume and planning target volume, respectively. The red region indicates the 95% isodose area of the prescribed dose (30 Gy)

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 > Discussion Top


There is no established treatment for primary MALT lymphoma of the bladder. Treatment options include surgery (including TUR-BT), radiotherapy, cytotoxic chemotherapy, targeted antibody therapy, or a combination of these modalities.[6] Radiotherapy is a good treatment option, with the advantage of organ preservation and low adverse events. Al-Maghrabi et al. investigated four patients who received radiotherapy for primary bladder lymphoma; all four patients were alive and relapse-free 2–13 years after treatment.[7] However, in patients of reproductive age, there is a risk of infertility secondary to definitive radiation to the bladder because of the proximity of nearby reproductive organs.[8] Similar to our case, TUR-BT combined with radiotherapy resulted in good treatment outcomes in another case report.[9] Lucioni et al. reported a case in which antibiotic therapy was effective for primary bladder MALT lymphoma bearing the chromosomal translocation t(11;18)(q21;q21), which is resistant to antibiotic therapy in gastric MALT lymphoma cases.[10]

In a retrospective analysis of data from patients with MALT lymphoma, there was no significant difference in the 10-year overall survival rate between the patients with gastric and nongastric MALT lymphoma (75% vs. 77%).[11] However, this analysis reported that the median time to progression of gastric MALT lymphoma was longer than that of nongastric MALT lymphoma (8.9 years vs. 4.9 years; P = 0.01). Careful follow-up is needed in our case.


 > Conclusion Top


Primary MALT lymphoma of the bladder is a rare malignancy. Our treatment, a combination of TUR-BT and radiotherapy, showed good treatment outcomes. Further accumulation of clinical data is needed to clarify the appropriate treatment strategy.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient (s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initial s will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
 > References Top

1.
Alderuccio JP, Florindez JA, Reis IM, Zhao W, Lossos IS. Treatments and outcomes in Stage I extranodal marginal zone lymphoma in the United States. Cancers (Basel) 2021;13:1803.  Back to cited text no. 1
    
2.
Karvounis E, Kappas I, Angelousi A, Makris GM, Kassi E. Mucosa-associated lymphoid tissue lymphoma of the thyroid gland: A systematic review of the literature. Eur Thyroid J 2020;9:11-8.  Back to cited text no. 2
    
3.
Ishibashi N, Nakanishi Y, Nishimaki H, Maebayashi T, Masuda S, Okada M. Bladder mucosa-associated lymphoid tissue lymphoma progressed from chronic cystitis along with a comparative genetic analysis during long-term follow-up: A case report. Transl Androl Urol 2021;10:3899-906.  Back to cited text no. 3
    
4.
Troppan K, Wenzl K, Neumeister P, Deutsch A. Molecular pathogenesis of MALT lymphoma. Gastroenterol Res Pract 2015;2015:102656.  Back to cited text no. 4
    
5.
Liu H, Ye H, Dogan A, Ranaldi R, Hamoudi RA, Bearzi I, et al. T (11;18)(q21;q21) is associated with advanced mucosa-associated lymphoid tissue lymphoma that expresses nuclear BCL10. Blood 2001;98:1182-7.  Back to cited text no. 5
    
6.
Lyapichev KA, Ivashkevich Y, Chernov Y, Chinenov D, Shpot E, Bessonov AA, et al. MALT lymphoma of the urinary bladder shows a dramatic female predominance, uneven geographic distribution, and possible infectious etiology. Res Rep Urol 2021;13:49-62.  Back to cited text no. 6
    
7.
Al-Maghrabi J, Kamel-Reid S, Jewett M, Gospodarowicz M, Wells W, Banerjee D. Primary low-grade B-cell lymphoma of mucosa-associated lymphoid tissue type arising in the urinary bladder: Report of 4 cases with molecular genetic analysis. Arch Pathol Lab Med 2001;125:332-6.  Back to cited text no. 7
    
8.
Mulder RL, Font-Gonzalez A, van Dulmen-den Broeder E, Quinn GP, Ginsberg JP, Loeffen EA, et al. Communication and ethical considerations for fertility preservation for patients with childhood, adolescent, and young adult cancer: Recommendations from the PanCareLIFE consortium and the international late effects of childhood cancer guideline harmonization group. Lancet Oncol 2021;22:e68-80.  Back to cited text no. 8
    
9.
Haddad-Lacle JE, Haddad CJ, Villas B. A rare urinary bladder tumour. BMJ Case Rep 2014;2014:bcr2013202994.  Back to cited text no. 9
    
10.
Lucioni M, Nicola M, Riboni R, Croci GA, Rattotti S, Gotti M, et al. Antibiotic therapy-induced remission of bladder mucosa-associated lymphoid tissue (MALT) lymphoma carrying t(11;18)(q21;q21) apoptosis inhibitor 2-MALT1. J Clin Oncol 2013;31:e304-6.  Back to cited text no. 10
    
11.
Thieblemont C, Bastion Y, Berger F, Rieux C, Salles G, Dumontet C, et al. Mucosa-associated lymphoid tissue gastrointestinal and nongastrointestinal lymphoma behavior: Analysis of 108 patients. J Clin Oncol 1997;15:1624-30.  Back to cited text no. 11
    


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