Primary T-cell Lymphoma of the Urinary Bladder Presenting ...

The Journal of International Medical Research
2011; 39: 2027 – 2032

Primary T-cell Lymphoma of the Urinary
Bladder Presenting with Haematuria and

Hydroureteronephrosis

L WANG1,a, ZZ CAO2,a AND L QI1

1Department of Urology, Xiangya Hospital, Changsha, China; 2Department of
Gynaecological Oncology, Hunan Provincial Tumour Hospital, Changsha, China

Primary bladder lymphoma, a rare form of nephrosis. A diagnosis of primary T-cell
non-Hodgkin’s lymphoma that is confined
to the urinary bladder, is usually of B-cell lymphoma of the urinary bladder was
origin. This report describes an extremely
rare case of primary T-cell lymphoma of the made on the basis of clinical, radiological
urinary bladder. A 45-year-old man
presented with haematuria, dysuria and and histological findings. The patient,
loin pain. Ultrasonography and computed
tomography showed a thickened left following transurethral resection, was
bladder wall and left hydrouretero-
treated with four cycles of CHOP

(cyclophosphamide, doxorubicin,

vincristine, prednisone) chemotherapy with

a good response and remains in clinical

remission 12 months after treatment.

KEY WORDS: LYMPHOMA; URINARY BLADDER; HAEMATURIA; HYDROURETERONEPHROSIS

Introduction and the treatment of a patient with primary
T-cell lymphoma of the bladder.
The incidence of lymphoma ranks among the
top 10 most prevalent malignant Case report
neoplasms.1 Extranodal lymphomas
comprise approximately 25 − 40% of all cases A 45-year-old man was referred to Xiangya
depending on the criteria used.2 The most Hospital, Changsha, China, in October 2009
common sites of primary extranodal with a 4-week history of intermittent
malignant lymphoma are the haematuria, dysuria and loin pain. Before
gastrointestinal tract, connective tissue and the onset of symptoms he had been in good
skin.3 Primary lymphoma of the urinary general health. Physical examination found
bladder is rare, accounting for < 1% of no organomegaly or lymphadenopathy.
urinary bladder neoplasms and 0.2% of all Routine haematological and biochemical
extranodal lymphoma cases.4 Most primary tests, including human immunodeficiency
bladder lymphomas are B-cell lymphomas.5,6 virus serology, were normal. An intravenous
The present case report describes the clinical pyelogram showed a lobulated filling defect
features, imaging and laboratory findings, on the left lateral wall of the bladder and
dilatation of the left upper urinary tract
aLong Wang and Zhenzhen Cao contributed equally to (Fig. 1). Ultrasonography and computed
this work.

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L Wang, ZZ Cao, L Qi
Primary T-cell lymphoma of the bladder

FIGURE 1: Intravenous pyelogram of a 45-year-old man presenting with a 4-week
history of intermittent haematuria, dysuria and loin pain, showing a left-sided lobular
defect of the urinary bladder and dilatation of the left upper urinary tract

tomography (CT) demonstrated an irregular cytokeratin antibody (CK-pan), epithelial
thickening of the left lateral wall of the membrane antigen, cytokeratin–low
bladder (Fig. 2) and left hydrouretero- molecular weight (CK-L), and cytokeratin–
nephrosis. Cystoscopy revealed a sessile high molecular weight (CK-H), ruling out the
haemorrhagic mass involving the left possibility of B-cell lymphoma and
ureteric orifice. Transurethral resection was carcinoma. Evaluation of the T-cell receptor
performed and histopathological gene rearrangement using polymerase chain
examination showed diffuse, dense reaction (PCR) showed that tumour cells
infiltration of the lamina propria by were monoclonal for the T-cell receptor γ
medium-sized atypical lymphocytes with chain gene (Fig. 4). Further investigations,
pleomorphic, hyperchromatic nuclei (Fig. including chest radiography, thoracic,
3A). The tumour cells were positive for CD3 abdominal and pelvic CT, and bone marrow
(Fig. 3B), CD56, CD45RO and TIA-1, which biopsy, demonstrated completely normal
were indicative of a T-cell-derived findings. The tumour was, therefore,
lymphoma. Immunohistochemical analysis diagnosed as stage I peripheral T-cell
showed that the tumour cells were negative lymphoma of the bladder.7
for CD20, CD79, smooth muscle actin, pan-
The patient underwent four 5-day cycles

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L Wang, ZZ Cao, L Qi
Primary T-cell lymphoma of the bladder

FIGURE 2: Computed tomography scan of a 45-year-old man presenting with a 4-
week history of intermittent haematuria, dysuria and loin pain, showing irregular
thickening of the left lateral wall of the bladder

of CHOP (cyclophosphamide 750 mg/m2, of terminal non-Hodgkin’s lymphoma cases,9
primary lymphomas of the urinary bladder
doxorubicin 50 mg/m2, vincristine 1.4 are very rare with just over 100 cases reported
since the original description in 1885.10
mg/m2, prednisone 100 mg) every 4 weeks. Clinical and radiographic examinations of
the present case excluded systemic
The lesions of the bladder and left urinary haematological malignancy and confirmed
a focused lymphoma of the bladder.
tract had nearly completely regressed at the
The most common subtypes of primary
end of the treatment. Subsequent follow-up bladder lymphoma are mucosa-associated
lymphoid tissue (MALT) lymphoma and
evaluations were performed at 3-month diffuse large B-cell lymphoma.5,6,8 The major
symptoms are intermittent haematuria,
intervals for 6 months and included clinical dysuria and urinary frequency. The tumour
occurs 6.5 times more frequently in women
examination, cystoscopy, chest than in men and the mean age of affected
patients is 64 years (range 20 − 85 years).11 A
roentgenography, CT of the abdomen and history of chronic cystitis has been shown to be
a preliminary feature in some cases of primary
pelvis, and routine blood tests. None of these bladder lymphoma, with approximately 20%
of patients presenting with such a history.12,13
follow-up procedures revealed evidence of
A review of the current English language
recurrence. The patient was still clinically medical literature identified only four cases
of primary T-cell lymphoma of the urinary
free of disease 12 months after transurethral bladder (including the present case) (Table
1).8,14,15 All four cases presented with
resection of the tumour.

Discussion

Malignant lymphoma of the urinary bladder
is a rare phenomenon that can occur in a
primary or secondary form. Primary bladder
lymphoma is defined as localized bladder
disease with no evidence of involvement of
surrounding tissue, lymphadenopathy on CT
of the chest, abdomen or pelvis, and no bone
marrow involvement.8 Although the urinary
bladder is secondarily involved in 10 − 20%

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L Wang, ZZ Cao, L Qi
Primary T-cell lymphoma of the bladder

A

B

FIGURE 3: Histopathological examination of a sessile haemorrhagic mass excised from
the bladder of a 45-year-old man presenting with a 4-week history of intermittent
haematuria, dysuria and loin pain, showing dense infiltration of atypical lymphocytes:
(A) haematoxylin and eosin staining; and (B) positive immunohistochemical staining
for the T-cell marker CD3 (scale bars 100 µm)

haematuria. Only one of the three previous immunohistochemical analyses were
published cases complained of dysuria,15 as negative for CD20, CD79, CK-pan, CK-L and
in the present case, and none demonstrated CK-H, ruling out the possibility of B-cell
hydroureteronephrosis. The tumour in the lymphoma and carcinoma. Genomic DNA
present case involved the left ureteral orifice was extracted and PCR of the clonal
and caused hydronephrosis of the left rearrangement of the T-cell receptor gene
kidney, which is an uncommon presentation revealed a monoclonal band for the T-cell
of primary lymphoma of the bladder. receptor γ gene on electrophoresis. Normal
mature T-cells express two mutually
Tissue biopsy and subsequent exclusive types of CD3-associated T-cell
immunohistochemistry were required to receptor heterodimers: the α/β T-cell
establish diagnosis because the clinical and heterodimer and the γ/δ T-cell heterodimer.
radiological features of this tumour are Peripheral T-cell lymphomas in which γ/δ T-
similar to those of urothelial carcinomas of cell receptors are expressed originate from
the bladder.16 Strong positive staining for γ/δ T-cells, a part of the innate immune
CD3 and CD56 indicated that the present system, and exhibit preferential homing to
case was a T-cell-derived lymphoma, while

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L Wang, ZZ Cao, L Qi
Primary T-cell lymphoma of the bladder

FIGURE 4: Genomic DNA, extracted from a sessile haemorrhagic mass excised from
the bladder of a 45-year-old man presenting with a 4-week history of intermittent
haematuria, dysuria and loin pain, was assessed by polymerase chain reaction for
clonal rearrangement of the T-cell receptor. The results showed rearrangement of the
T-cell receptor γ chain gene as a monoclonal band on gel electrophoresis (lane 1,
marker; lane 2, negative control; lane 3, positive control; lane 4, patient sample)

TABLE 1:
Summary of the present case and three cases of primary bladder T-cell lymphomas
reported in the English language medical literature

Reference Age/sex Presenting symptoms Treatment Outcome

Hughes et al.8 27/male Haematuria, left iliac Surgical excision; Alive (7 years); no
fossa pain chemotherapy: evidence of disease

Choi et al.15 30/female Haematuria, dysuria CHOP (×4) Died
Mourad et al.14 52/male Haematuria, suprapubic None Not available

pain Chemotherapy: Alive (1 year); no
Present case 45/male Haematuria, dysuria, CHOP evidence of disease

loin pain transurethral
resection;

chemotherapy:
CHOP (×4)

CHOP, cyclophosphamide, doxorubicin, vincristine, prednisone.

the respiratory or gastrointestinal tract treatment options for primary bladder
mucosa.17 It is unusual for the urinary lymphoma.6 Patient characteristics, such as
bladder to be affected by γ/δ T-cell age, tumour grade and the coexistence of
lymphomas and the significance of this systemic disease, should be considered when
finding remains to be evaluated. selecting treatment modalities. Chemotherapy
is currently favoured because it also treats
As a result of the rarity of the disease, the undetectable early systemic disease. Surgical
optimal treatment for primary bladder approaches can be beneficial for patients with
lymphomas is not yet known. Chemotherapy, irritative urinary symptoms. Primary
radiotherapy and surgery, as well as malignant lymphoma of the bladder usually
combinations of these therapies, are all

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L Wang, ZZ Cao, L Qi
Primary T-cell lymphoma of the bladder

exhibits a silent course with a favourable not been described previously. This sort of
prognosis.6 Peripheral T-cell lymphoma, presentation can mimic other urothelial
however, is known to have a worse prognosis carcinomas of the bladder and presents a
than B-cell lymphoma.17 The prognosis is diagnostic challenge. Tissue biopsy with
associated with the tumour grade and stage at thorough immunohistochemistry and
presentation. Following transurethral analysis of T-cell receptor gene
resection and four cycles of chemotherapy rearrangements are required to make a
there has been no reappearance of tumour or conclusive diagnosis.
its related symptoms in the present case.
Conflicts of interest
Primary T-cell lymphoma of the urinary
bladder presenting simultaneously with The authors had no conflicts of interest to
haematuria and hydroureteronephrosis has declare in relation to this article.

• Received for publication 29 March 2011 • Accepted subject to revision 12 April 2011
• Revised accepted 9 August 2011

Copyright © 2011 Field House Publishing LLP

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Author’s address for correspondence

Dr Lin Qi

Department of Urology, Xiangya Hospital, Central South University, 87 Xiangya Road,

Changsha, Hunan 410008, China.

E-mail: [email protected]

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