Bladder - Lymphoma (primary)

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Lymphoma (primary)

Reviewers: Gillian Levy, M.D., Yale Medical Center (see Reviewers page)
Revised: 24 April 2011, last major update April 2011
Copyright: (c) 2003-2011, PathologyOutlines.com, Inc.

Definition
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● Either primary (rare, originating in bladder with no known lymphoma elsewhere) or secondary (much more common)

Epidemiology
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● Female predominance, most cases are in middle aged women
● Secondary bladder involvement occurs in 10-25% of leukemias / lymphomas and in advanced stage systemic lymphoma
● Rare as primary; <100 cases
● MALT is most common subtype in bladder; typically affects adults > 60 years, 75% female
● Diffuse large B cell lymphoma is also common, and may arise from transformation of MALT (J Clin Pathol 2000;53:458)

Sites
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● Bladder, lower ureteral tract

Clinical features
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● Comprises 5% of non-urothelial tumors of urothelial tract
● Long median survival for either primary lymphoma of bladder or lymphoma with initial presentation in bladder but other coexisting disease (Am J Surg Pathol 1997;21:1324)
● Recurrent lymphoma in bladder is associated with widely disseminated disease and poor prognosis
● Presents with gross hematruia, dysuria, urinary frequency, nocturia, and abdominal or back pain

● Low grade MALT lymphoma: most common lymphoma subtype in bladder; much more common as secondary tumor than primary tumor; history of chronic cystitis common (Arch Pathol Lab Med 2001;125:332)

Prognostic factors
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● Histologic subtype, stage

Case reports
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● 52 year old man with schistosomiasis and T cell lymphoma (Am J Surg Pathol 1998;22:373)
● 67 year old woman with EBV+ B cell lymphoproliferative disorder (Indian J Urol 2009;25:129)
● 72 year old woman with exophytic mass at bladder base and MALT lymphoma (Mod Pathol 1993;6:145)
● 75 year old woman with diffuse large B cell lymphoma (Intern Med 2009;48:1403)
● Elderly woman with B cell lymphoma with signet-ring cells (Arch Pathol Lab Med 1991;115:635)

Treatment
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● MALT: radiation therapy, usually no recurrences

Radiological imaging
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● Submucosal masses: 70% of cases are solitary mass, 20% of cases are multiple masses, and 10% of cases show diffuse wall thickening

Gross description (Macroscopy)
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● Discrete tumors, usually large and centered in dome or lateral walls

Micro description (Histopathology)
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● MALT: sheets of low grade, uniform cells that surround and separate, but don’t destroy muscle fascicles

Micro images
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MALT:

Tumor cells separate and surround rather than destroy the smooth muscle fascicles of the bladder wall


Bland lymphocytes fill lamina propria but don’t involve urothelium

Lymphocytes permeate muscle fascicles

Monocytoid cells with clear cytoplasm

Fig 1: MALT involving lamina propria; fig 2: focal lymphoepithelial lesions in area of cystitis glandularis

EBV+ B cell lymphoproliferative disorder:


Left: diffuse infiltrate of atypical lymphocytes; right: CD20+


Left: CD30+; right: EBER+

Cytology description
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● MALT: monomorphic small to medium sized lymphocytes

Positive stains
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● Varies by subtype of lymphoma; B cell lymphomas are CD20+
● MALT lymphoma: CD20+, CD19+, CD5-, CD23-, CD10-, CD11c-, FMC7+

Negative stains
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● Pan-keratin, vimentin, CK20, CK7

Molecular/cytogenetics description
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● MALT Lymphoma: t(11;18) (q21:21)

Differential diagnosis
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● Urothelial carcinoma with prominent lymphoid infiltrate: see Am J Surg Pathol 1991;15:569
● Undifferentiated carcinoma

End of Bladder > Other tumors > Lymphoma

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