Case Reports in Hematology

Case Reports in Hematology / 2015 / Article

Case Report | Open Access

Volume 2015 |Article ID 934374 | 7 pages | https://doi.org/10.1155/2015/934374

MALT Lymphoma of the Bladder: A Case Report and Review of the Literature

Academic Editor: Marie-Christine Kyrtsonis
Received09 Jul 2015
Accepted12 Aug 2015
Published31 Aug 2015

Abstract

The presentation of a MALT lymphoma in the bladder is exceedingly rare. Furthermore, the optimal treatment of primary MALT confined to the bladder remains to be defined. Here, we report a case of a 65-year-old female with primary MALT lymphoma treated with definitive radiation therapy. The patient received a total dose of 30 Gy in 20 equal daily fractions to the bladder and tolerated the treatment well. In addition, we have extensively reviewed the relevant literature to better define the optimal management of this rare disease. In conclusion, primary MALT lymphoma of the bladder represents a rare malignancy with excellent prognosis if detected at an early stage. For early stage disease, definitive radiation represents an excellent treatment modality with a minimal side-effect profile.

1. Introduction

The majority of bladder cancers are epithelial in origin [1, 2]. Lymphomas of the urinary bladder are exceedingly rare and can be divided into (i) primary, a rare lymphoma arising in the urinary bladder with no evidence of lymphoma elsewhere, or (ii) secondary lymphoma of the urinary bladder associated with lymphoma at an extra vesicle site [3, 4]. Primary malignant lymphoma of the bladder accounts for less than 1% of neoplasms unlike secondary lymphoma, which is much more common [1, 2]. Of primary lymphomas of the bladder, mucosa associated lymphoid tissue lymphoma, or MALT, is the most prevalent histological subtype. The optimal treatment of primary MALT confined to the bladder remains to be defined. Here, we report a patient treated with definitive radiation and review the relevant literature to better define the optimal management of this rare disease.

2. Case Report

A 65-year-old female presented to her gynecologist after she noted a two-week history of spotting on toilet paper after urinating. She was referred to a urologist for further investigation of the bladder lesion. A transurethral resection of the bladder tumor (TURBT), with resection of the posterior bladder wall, right bladder wall, and bladder neck, was performed at an outside hospital. Initial pathology based on H&E stain and immunohistochemistry favored extra nodal marginal zone lymphoma with follicular colonization, with cells positive for CD20 and PAX-5 and negative for CD5 and CD10 (Figure 1). Laboratory evaluation including LDH, B2-microglobulin, serum immunofixation, and protein electrophoresis was all within normal limits. Subsequently, the patient underwent a PET/CT and bone marrow biopsy, and there was no evidence of any extra vesicular disease.

The patient was referred to radiation oncology to discuss the role of definitive radiation in her treatment regimen. Magnetic resonance imaging and repeat cystoscopy were recommended and performed to assess the presence of residual disease and were both negative. A well-healed biopsy area was noted on cystoscopy consistent with the site of original lesion. At this time, the patient’s hematuria was resolved and she denied any weight loss, fatigue, night sweats, or fevers. The patient had no history of recurrent sexually transmitted diseases. There was no lymphadenopathy, organomegaly, or abnormal findings on physical examination.

Based on patient’s lack of symptoms, negative imaging, and negative repeat cystoscopy, the patient was offered a course of close observation with serial cystoscopic evaluations versus definitive radiation given that the presence of microscopic disease could not be ruled out. She elected to proceed with radiation therapy. The patient received a total dose of 30 Gy in 20 equal daily fractions with a 4-field 3D-CRT plan utilizing PH16 MV photons. The adjacent normal structures were shielded with a multileaf collimator (MLC) (Figure 2). The patient tolerated radiation treatment well. She had no gastrointestinal, urinary, or gynecological toxicities during treatment and at short interval follow-up. Repeat evaluation 3 months following radiation with PET/CT revealed no evidence of disease and urine cytology was also negative.

3. Discussion

Primary lymphoma of the bladder is a rare malignancy, with limited literature to guide therapy. The first ever reported case of bladder lymphoma was reported in the literature by Eve and Chaffey in 1885 [48]. There have been less than 100 cases described in the literature since [4, 9, 10]. The disease typically presents in the 6th decade of life with slight predominance in females [6, 10]. Since lymphoid tissue is not found in the urinary bladder, chronic inflammation is postulated as the origin. As such, most patients present with symptoms of chronic cystitis [9]. However, similar to our patient, there have been many reported cases in which chronic cystitis and histological evidence of inflammation are lacking [4, 6, 11, 12]. The most common symptoms of lymphoma of the urinary bladder include weight loss, fatigue, hematuria, dysuria, nocturia, urinary frequency, and suprapubic or abdominal pain [4, 11, 12]. Table 1 contains a summary of basic patient demographics, presenting symptoms, treatments, and outcomes of all reported cases of primary MALT lymphoma of the bladder.


AuthorAgeSexPresenting symptomTreatmentFollow-up (Months)Outcome

Hughes et al., [22]81FemaleHematuriaDiathermy TURBT12NED
Ando et al., [16]77FemaleUrinary retentionTURBT36NED
Kempton et al., [23]64FemalePartial cystectomy144Died of unknown cause
Fernandez Acenero et al., [24]73FemaleDysuria, Back painChemotherapy8Died of unknown cause
Fernandez Acenero et al., [24]50FemaleFeverChemotherapy60NED
Fernandez Acenero et al., [24]75FemaleHematuria, dysuria and Breast carcinomaChemotherapy9NED
Gallardo et al., [25]70FemaleHematuria, Dysuria, Malaise and weight lossChemotherapy24NED
Morita et al., [26]68Femalepersistent interstitial cystitisChemotherapy [Rituximab]NED
Wazait et al., [27]65FemaleHematuria, dysuria and recurrent UTI’sChemotherapy [CHOP]36NED
Hughes et al., [22]77MaleHematuriaChemotherapy [ChID]48NED
Hughes et al., [22]28MaleHematuriaChemotherapy [ChIVP]120NED
Kakuta et al., [17]84FemaleGeneral fatigue and weight lossChemotherapy [R-CHOP]
Sen et al., [28]31FemaleIncidental finding - PregnancyPostpartum Chemotherapy
Kempton et al., [23]79FemaleRadiation24Died of Myocardial infarction
Al-Maghrabi et al., [10]64FemaleHematuria, and recurrent UTIRadiation156NED
Al-Maghrabi et al., [10]69FemaleTreatment resistant UTIRadiation60NED
Al-Maghrabi et al., [10]72FemaleHematuria and recurrent UTIRadiation36NED
Al-Maghrabi et al., [10]62MaleHematuriaRadiation24NED
Tsang et al., [29]RadiationNED
Tsang et al., [29]RadiationNED
Tsang et al., [29]RadiationNED
Hughes et al., [22]76FemaleHematuriaRadiation24NED
Hughes et al., [22]66FemaleRecurrent UTIRadiationDeceased
Takahara et al., [30]85FemaleHematuriaRadiationNED
Hatano et al., [15]84FemaleHematuria and recurrent UTIRadiation12NED
Kempton et al., [23]73FemaleFulguration and Radiation120Died of unknown cause
Kempton et al., [23]27FemaleTumor resection and Radiation480Died of fibrosarcoma
Kempton et al., [23]45MaleSegmental resection and Radiation312Died of unknown cause
Kempton et al., [23]50FemaleSegmental resection and Radiation240NED
Ueno et al., [31]64FemaleIncidental findingTURBT and Radiation19Recurrence Stomach
Haddad-lacle et al., [32]54MaleLow back pain, incidental bladder massTURBT and Radiation36NED
Wazait et al., [27]70FemaleHematuriaTURBT-1 yr recurrence, Chemotherapy on recurrence60NED
Szopiński et al., [33]17FemaleIncidental findingTURBT and Chemotherapy24NED
Maninderpal et al., [34]65FemaleChronic suprapubic mass, nausea and feeling unwellTURBT and Chemotherapy3Died of Sepsis
Matsuda et al., [3]78FemaleRefractory Cystitis and renal dysfunctionTURBT and Chemotherapy [rituximab]
Kawakami et al., [35]27MaleRadiation and Chemotherapy [doxorubicin]18NED
Tasu et al., [36]75FemaleHematuriaRadiation and Chemotherapy [cyclophosphamide]36NED
Painemal et al., [37]70FemaleHematuria, Dysuria, Malaise and weight lossRadiation and Chemotherapy48NED
Hughes et al., [22]82FemaleHematuriaRadiation + Chemotherapy [ChIVP]Deceased
Terasaki et al., [19]64FemaleGeneral malaise and anemiaRadiation and Chemotherapy [Rituximab]14NED
Mizuno et al., [38]72FemaleHematuria and recurrent cystitisTURBT, Radiation and Chemotherapy [rituximab]
Bacalja et al., [39]48MaleIncidental findingTURBT, Radiation and Chemotherapy [R-CHOP]5Remission
Mayer et al., [40]70MaleBladder mass with scalp metastasisChemotherapy and Radiation for scalp and brain14Died of Brain Metastasis
van den Bosch et al., [41]59MaleHematuriatriple therapy [patient denied radiation]36NED
Fujimura et al., [42]69FemaleHematuria and anemiaAntibiotics and H. pylori therapy24NED
Terada, [43]88FemaleHematuriaAntibiotics6Markedly reduced tumor size
Lucioni et al., [20]72FemalePersistent dysuriaAntibiotics6NED
Bates et al., [11]66FemaleBladder mass12NED
Bates et al., [11]79FemaleHematuria
Bates et al., [11]59FemaleUntreated solid necrotic tumor36NED
Kröber et al., [44]57MaleObstructive dysuria
Takahashi et al., [30]71FemaleHematuria

Authors do not have access to this information.
CHOP: cyclophosphamide, hydroxydaunorubicin, vincristine, and prednisone.
R-CHOP: cyclophosphamide, hydroxydaunorubicin, vincristine, prednisone, and rituximab.
ChlVP: chlorambucil, vincristine, and prednisolone.
TURBT: transurethral resection of bladder tumors.
NED: no evidence of disease.

There are many treatment options for nongastric MALT lymphomas: (i) observation (based on factors such as patient age, risk factors, and tumor grade, observation might be the best option [11]); (ii) surgery (complete excision or biopsy) (MALT lymphomas that are unifocal can be partially or completely removed with procedures like TURBT [11]); (iii) radiation (radiation, when given alone or after an excisional biopsy, has shown excellent local control and improved overall disease-free survival [10, 1315]); (iv) chemotherapy (usually used when a patient presents with systemic involvement or secondary lymphoma of the bladder [1618]); (v) targeted antibody therapy (anti-CD20 antibody (rituximab) has been used along with other modalities in systemic lymphomas [17, 19]); (vi) antibiotics (they are usually used in cases where there is a known bacterial origin such as H. pylori in the stomach). There have been rare cases where antibiotics were used for MALT lymphomas of the bladder [20].

The presentation of bladder MALT lymphoma is exceedingly rare; however, MALT lymphomas at other sites are common, especially in the GI tract, salivary gland, lung, Waldeyer’s ring, ocular adnexa, skin, thyroid, and breast. These lymphomas are highly radiosensitive. For localized disease, radiotherapy is the most appropriate treatment for organ preservation. It should be noted that, in patients of reproductive age, there is a risk of infertility with definitive radiation to the bladder secondary to the proximity of nearby reproductive organs. In these cases, maximal resection with TURBT may be the best treatment option when fertility is of concern. Chemotherapy and rituximab are reserved for secondary, recurrent, or disseminated disease [1618, 21]. Al-Maghrabi et al. in 2001 identified four patients who received low dose radiotherapy for Stage IAE primary lymphoma of the bladder in a 30-year retrospective study. All four patients are alive and recurrence-free 2–13 years after treatment [10].

When considering radiation as a definitive monotherapy, staging becomes of utmost importance. 18F-FDG PET/CT, pelvic MRI, and bone marrow biopsy are used for initial disease staging and to rule out disseminated disease [1, 2, 10, 12]. As there was no evidence of disease in our patient’s imaging work-up and post-TURBT cystoscopy, she was presented with observation with cystoscopy at 3-4-month intervals versus radiation therapy with less frequent cystoscopy and imaging. The optimal follow-up strategy for patients with lymphoma of the bladder remains unknown. In our practice, once the patient achieves complete response on posttreatment imaging, no further imaging is indicated. Urine cytology should be performed at each visit and annual cystoscopy should be performed for the first 2-3 years.

In conclusion, primary MALT lymphoma of the bladder represents a rare malignancy with excellent prognosis if detected at an early stage. For early stage disease, definitive radiation represents an excellent treatment modality with a minimal side-effect profile.

Conflict of Interests

The authors declare that there is no conflict of interests regarding the publication of this paper.

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Copyright © 2015 Prashant Vempati et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

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