Lymphomatous involvement of the kidney is often seen as a part of disseminated disease. Characteristics of disease are poor prognosis and survival of less than a year after diagnosis. The primary renal lymphoma is very rare, is usually part of general disease, is difficult to diagnostic, and has a poor

prognosis.

Noncharacteristic solitary renal masses need to be differentiated from renal cell carcinoma. We present a

73-year-old man with a solitary renal mass treated with partial nephrectomy and histologic diagnosed with a primary lymphoma of the kidney. In the preventive diagnosis, conventional radiology is unsuitable,

contrast-enhanced renal ultrasound contributes to the diagnosis, and primary renal biopsy is useful.

We reviewed the medical literature and discuss primary renal lymphoma, a rare disease with poor prognosis,

whose treatment after postsurgical diagnosis based on chemotherapy with R-CHOP (rituximab plus cyclophosphamide/doxorubicin/vincristine/prednisone)

Alvaro, R

Brancato, T

D'Ascenzo, R

Nupieri, P

Orsolini, G

Paulis, G

Archivio della Ricerca - Università di Roma Tor vergata

This article appeared in a journal published by Elsevier. The attached
copy is furnished to the author for internal non-commercial research
and education use, including for instruction at the authors institution
and sharing with colleagues.
Other uses, including reproduction and distribution, or selling or
licensing copies, or posting to personal, institutional or third party
websites are prohibited.
In most cases authors are permitted to post their version of the
article (e.g. in Word or Tex form) to their personal website or
institutional repository. Authors requiring further information
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encouraged to visit:
http://www.elsevier.com/copyright
Author's personal copy
Primary Lymphoma of the Kidney: Case Report
and Review of Literature
Tommaso Brancato,1 Rosaria Alvaro,2 Gianni Paulis,1 Pietro Nupieri,1
Roberto D’Ascenzo,1 Giuseppe Orsolini1
Introduction
Incidence of non-Hodgkin lymphoma (NHL) has progressively
increased to 150% compared with the past century.1 The evolution
of this disease shows a variation of incidences and a significant in-
crease in patients older than the normal bimodal pattern in which the
age group most affected is the young adult, predominantly male,
population. The incidence of cases of renal involvement in NHL has
been reported as between 2.7% and 6%.2 Compared with a second-
ary involvement, primary renal lymphoma is exceptional and repre-
sents about 1% of the all renal tumors.3
The existence of a primary lymphatic renal disease (PRL) has been
discussed, because the kidney does not contain lymphatic tissue and it is
unclear how the disease may develop. Our hypothesis is the following:
the presence of lymphatic tissue lymphatic vessels of the hilum or capsule
or intensive recall of the B lymphocytes in the parenchyma in response to
a persistent inflammation. There are approximately 100 cases described
in the medical literature.4 The term PRL is then applied to a renal
localization in the absence of clinical manifestations of lymphatic disease
in other organs and may also be defined as a NHL that starts. We present
the case came to our observation and review of the literature.
Case Report
An 73-year-old white male patient came to our attention for ul-
trasound documented bilateral renal cysts. Results of blood tests
showed only a slight lymphocytopenia (15.8% of lymphocytes) and
neutrophilic leukocytosis (76.8% of leukocytes). Hepatitis serolo-
gies, lactate dehydrogenase, and chest radiographs were negative.
Due to the thickened wall cyst with a diagnostic doubt, the patient
underwent contrast-enhanced renal ultrasound (Sonovue Bracco Di-
agnostics Inc, Princeton, NJ) that confirm, in the third lower right
kidney with anterior development, a irregular appearance formation
of 52 mm, partly solid. Administration of contrast agent showed
enhancement of the wall, and remains avascular the central portion.
The patient then underwent a contrast computed tomography (CT)
of the abdomen, which confirmed the presence of lesion average 52
mm diameter, moderately thickened walls at baseline, irregular and
hyperdense, and homogeneous content. After administration of con-
trast, there was no significant enhancement of the wall of lesion or
parietal tokens are shown. CT of the chest, abdomen, and pelvis
showed no further histologic localization of disease; also, a bone
marrow biopsy showed a bone marrow free of significant histologic
changes. Renal tumor was suspected and therefore we do not do a
positron emission tomography, moreover, this examination cannot
1Department Of Urology, Regina Apostolorum Hospital, Albano Laziale – Rome, Italy
2Department of Health, Tor Vergata University, Rome, Italy
Submitted: Jul 11, 2011; Revised: Dec 2, 2011; Accepted: Dec 11, 2011
Address for correspondence: Tommaso Brancato, MD, Department of Urology, Regina
Apostolorum Hospital, 50 Via S. Francesco, Albano Laziale, Rome 00040, Italy
Fax:390693298506; e-mail contact: tommaso.brancato@gmail.com
Clinical Practice Points
● Lymphomatous involvement of the kidney is often
seen as a part of disseminated disease. Character-
istics of disease are poor prognosis and survival of
less than a year after diagnosis. The primary renal
lymphoma is very rare, is usually part of general
disease, is difficult to diagnostic, and has a poor
prognosis.
● Noncharacteristic solitary renal masses need to be
differentiated from renal cell carcinoma. We present a
73-year-old man with a solitary renal mass treated
with partial nephrectomy and histologic diagnosed
with a primary lymphoma of the kidney. In the preven-
tive diagnosis, conventional radiology is unsuitable,
contrast-enhanced renal ultrasound contributes to the
diagnosis, and primary renal biopsy is useful.
● We reviewed the medical literature and discuss pri-
mary renal lymphoma, a rare disease with poor prog-
nosis, whose treatment after postsurgical diagnosis
based on chemotherapy with R-CHOP (rituximab plus
cyclophosphamide/doxorubicin/vincristine/prednisone).
Clinical Genitourinary Cancer, Vol. 10, No. 1, 60-2 © 2012 Elsevier Inc. All rights reserved.
Keywords: Imaging, Non-Hodgkin lymphoma, Partial nephrectomy, Primary renal lymphoma, Renal cell carcinoma
Case Report
60 Clinical Genitourinary Cancer March 2012
1558-7673/$ - see frontmatter © 2012 Elsevier Inc. All rights reserved.
doi: 10.1016/j.clgc.2011.12.001
Author's personal copy
evaluate well the kidney because renal fluorine-18 fluorodeoxyglu-
cose excretion interferes with kidney imaging.
A preliminary characterization of lesion biopsy was not performed
due to the risk of seeding in this malignancy case. Due to the reduced
renal function (Glomerular filtration rate 59 mL/min), we decided to
perform neprhon-sparing surgery enucleoresection. The patient was
treated with enucleoresection with renal warm ischemia for 22 min-
utes. On histologic examination, macroscopic formation had a max-
imum diameter of 6.8-cm, a thick fibrous wall that contained a large
amount of necrotic dampish material, yellowish-gray in color. A
microscopic examination documented non-Hodgkin anaplastic
large cell B extensively necrotic and capsule (Figure 1). The cells were
positive for CD 20 (Figure 2), PAX 5, Bcl 2, CD 30, vimen-
tin, and CD 45, and were negative for CD3–, CKMNF116–,
CK7–, CD10–, EMA–, CD56–, desmin–, -Myod1, Rod–, and
CD31–.
Discussion
PRL is defined as a NHL that starts in the parenchyma and does not
show invasion into adjacent lymph nodes. It is very rare and the diagno-
sis of PRL is difficult.5 Lymphomas are malignant neoplasms character-
ized by the proliferation of cells in the lymphoid tissue. Lymphomatous
involvement of the genitourinary tract occurs in 3%-6% of cases and is
most common in the testis. The primary lymphoma of the kidney is a
unique disease entity, because the kidney does not contain lymphatic
tissue. There is no evidence that the disease is the primary or initial
manifestation of systemic disease rapidly aggressive. It has been sug-
gested that the lymphoma may originate from the renal sinus lymphatics
or lymph vessels located near the renal capsule and form cords that enter
into renal parenchyma, or follow chronic inflammation (such as chronic
pyelonephritis, Sjogren’s Syndrome, Systemic Lupus Erythematosus,
Epstein Barr Virus of mononucleosis).6 Many of the documented cases
of renal lymphoma are secondary, and postmortem studies have found
that approximately 50% of patients who died of NHL had renal involve-
ment. PRL most commonly affects older male subjects, those older than
60 years (mean age at diagnosis, 64 years), frequently unilateral, rarely
bilateral.7,8 Large B-cell lymphoma is the most common histologic type,
in up to approximately 30% of NHL, and is among the most clinically
aggressive lymphoma. The clinical manifestations are similar to those of
other renal neoplasms also may present with: proteinuria, nephrotic
syndrome, kidney failure. It is very aggressive, with rapid systemic dis-
semination. Survival is extremely low, approximately 75% of patients
die within the first year after surgery.6 The final prognosis can be im-
proved by early diagnosis and systemic chemotherapy. There are several
options for the diagnostic evaluation of a possible involvement of the
kidney, including renal ultrasonography, intravenous urography, CT,
magnetic resonance imaging, and nuclear medicine.7 The ultrasound
contrast agent is the mode of choice, whereas normal CT with contrast
techniques have not been a valuable diagnostic aid due to the extreme
variability of findings that may be encountered in cases of renal
lymphoma.
Conclusions
Overall, the prognosis of patients with renal lymphoma is less than
1 year.5 The PRL is considered a systemic disease with renal mani-
festation, even without evidence of extrarenal locations. The total
nephrectomy is not indicated, especially if it was possible to make a
preoperative diagnosis.6 Patients with atypical findings suspected for
renal cell carcinoma should undergo a percutaneous renal biopsy,
which has a sensitivity and a specificity of 70% -92% and 100%,
respectively.5,6 Although renal lymphoma is often part of a general-
ized process, our case appears to be limited to the kidney, so-called
stage IE diffuse large B-cell lymphoma. This early-stage aggressive
lymphoma, may be highly curable with CHOP (cyclophosphamide,
doxorubicin, vincristine [Oncovin], and prednisone] plus rituximab,
based on the revised International Prognostic Index of Sehn et al,9
but only 41%-53% of patients with advanced disease are alive and
disease free at a median follow-up of 3 years, especially when there is
an overexpression of Bcl 2.5-9
Figure 1 (A) Atrophic Renal Tubules. (B) Infiltration of
Lymphoplasmacytic Cells. (C) Fibrous Capsule.
(D) Tumor Cells. (E) Necrosis. Renal Lymphoma
(Hematoxylin and Eosin)  30
A
B
C
D
E
Figure 2 Immunohistochemistry of Renal Lymphoma:
coloration for CD20 Lymphocytes  400
Clinical Genitourinary Cancer March 2012 61
Author's personal copy
Disclosure
The authors have stated that they have no conflicts of interest.
References
1. SEER Registry (surveillance epidemiology and end results). Available at: http://
www.nci.nih.gov.Accessed: March 4, 2011.
2. Omer HA, Hussein MR. Primary renal lymphoma. Nephrology (Carlton) 2007; 12:
314-5.
3. Stallone G, Infante B, Manno C, et al. Primary renal lymphoma does exist: case report
and review of the literature. J Nephrol 2000; 13:367-72.
4. Besso L, Quercia AD, Daidola G, et al. Il coinvolgimento renale nei linfomi. G Ital
Nefrol 2010; 27(suppl 50):34-9.
5. Pervez H, Shaikh M, Potti A, et al. Uncommon presentation of non-Hodgkin’s
lymphoma. Case 3. Primary renal lymphoma. J Clin Oncol 2003; 21:567-9.
6. Cyriac S, Rejiv R, Shirley S, et al. Primary renal lymphoma mimicking renal cell
carcinoma. Indian J Urol 2010; 26:441-3.
7. Pinggera GM, Peschel R, Buttazzoni A, et al. A possible case of primary renal lym-
phoma: a case report. Cases J 2009; 2:6233 [Web site]. Available at: http://www.
casesjournal.com/casesjournal/article/view/6233. Accessed: March 1, 2011.
8. Vázquez AF, Sánchez RC, Vicente Prados FJ, et al. Primary renal lymphoma: report
of three new cases and literature review. Arch Esp Urol 2009; 62:461-5.
9. Sehn LH, Berry B, Chhanabhai M, et al. The revised International Prognostic
Index (R-IPI) is a better predictor of outcome than the standard IPI for patients
with diffuse large B-cell lymphoma treated with R-CHOP. Blood 2007; 109:
1857-61.
Primary Lymphoma of the Kidney
62 Clinical Genitourinary Cancer March 2012


Primary Lymphoma of the Kidney: Case Report

and Review of Literature

Lymphomatous involvement of the kidney is often seen as a part of disseminated disease. Characteristics of disease are poor prognosis and survival of less than a year after diagnosis. The primary renal lymphoma is very rare, is usually part of general disease, is difficult to diagnostic, and has a poor
prognosis.
Noncharacteristic solitary renal masses need to be differentiated from renal cell carcinoma. We present a
73-year-old man with a solitary renal mass treated with partial nephrectomy and histologic diagnosed with a primary lymphoma of the kidney. In the preventive diagnosis, conventional radiology is unsuitable,
contrast-enhanced renal ultrasound contributes to the diagnosis, and primary renal biopsy is useful.
We reviewed the medical literature and discuss primary renal lymphoma, a rare disease with poor prognosis,
whose treatment after postsurgical diagnosis based on chemotherapy with R-CHOP (rituximab plus cyclophosphamide/doxorubicin/vincristine/prednisone)

Orsolini, G.

ALVARO, ROSARIA

This article appeared in a journal published by Elsevier. The attachedcopy is furnished to the author for internal non-commercial researchand education use, including for instruction at the authors institutionand sharing with colleagues.Other uses, including reproduction and distribution, or selling orlicensing copies, or posting to personal, institutional or third partywebsites are prohibited.In most cases authors are permitted to post their version of thearticle (e.g. in Word or Tex form) to their personal website orinstitutional repository. Authors requiring further informationregarding Elsevier’s archiving and manuscript policies areencouraged to visit:http://www.elsevier.com/copyrightAuthor's personal copyPrimary Lymphoma of the Kidney: Case Reportand Review of LiteratureTommaso Brancato,1 Rosaria Alvaro,2 Gianni Paulis,1 Pietro Nupieri,1Roberto D’Ascenzo,1 Giuseppe Orsolini1IntroductionIncidence of non-Hodgkin lymphoma (NHL) has progressivelyincreased to 150% compared with the past century.1 The evolutionof this disease shows a variation of incidences and a significant in-crease in patients older than the normal bimodal pattern in which theage group most affected is the young adult, predominantly male,population. The incidence of cases of renal involvement in NHL hasbeen reported as between 2.7% and 6%.2 Compared with a second-ary involvement, primary renal lymphoma is exceptional and repre-sents about 1% of the all renal tumors.3The existence of a primary lymphatic renal disease (PRL) has beendiscussed, because the kidney does not contain lymphatic tissue and it isunclear how the disease may develop. Our hypothesis is the following:the presence of lymphatic tissue lymphatic vessels of the hilum or capsuleor intensive recall of the B lymphocytes in the parenchyma in response toa persistent inflammation. There are approximately 100 cases describedin the medical literature.4 The term PRL is then applied to a renallocalization in the absence of clinical manifestations of lymphatic diseasein other organs and may also be defined as a NHL that starts. We presentthe case came to our observation and review of the literature.Case ReportAn 73-year-old white male patient came to our attention for ul-trasound documented bilateral renal cysts. Results of blood testsshowed only a slight lymphocytopenia (15.8% of lymphocytes) andneutrophilic leukocytosis (76.8% of leukocytes). Hepatitis serolo-gies, lactate dehydrogenase, and chest radiographs were negative.Due to the thickened wall cyst with a diagnostic doubt, the patientunderwent contrast-enhanced renal ultrasound (Sonovue Bracco Di-agnostics Inc, Princeton, NJ) that confirm, in the third lower rightkidney with anterior development, a irregular appearance formationof 52 mm, partly solid. Administration of contrast agent showedenhancement of the wall, and remains avascular the central portion.The patient then underwent a contrast computed tomography (CT)of the abdomen, which confirmed the presence of lesion average 52mm diameter, moderately thickened walls at baseline, irregular andhyperdense, and homogeneous content. After administration of con-trast, there was no significant enhancement of the wall of lesion orparietal tokens are shown. CT of the chest, abdomen, and pelvisshowed no further histologic localization of disease; also, a bonemarrow biopsy showed a bone marrow free of significant histologicchanges. Renal tumor was suspected and therefore we do not do apositron emission tomography, moreover, this examination cannot1Department Of Urology, Regina Apostolorum Hospital, Albano Laziale – Rome, Italy2Department of Health, Tor Vergata University, Rome, ItalySubmitted: Jul 11, 2011; Revised: Dec 2, 2011; Accepted: Dec 11, 2011Address for correspondence: Tommaso Brancato, MD, Department of Urology, ReginaApostolorum Hospital, 50 Via S. Francesco, Albano Laziale, Rome 00040, ItalyFax: 390693298506; e-mail contact: tommaso.brancato@gmail.comClinical Practice Points● Lymphomatous involvement of the kidney is oftenseen as a part of disseminated disease. Character-istics of disease are poor prognosis and survival ofless than a year after diagnosis. The primary renallymphoma is very rare, is usually part of generaldisease, is difficult to diagnostic, and has a poorprognosis.● Noncharacteristic solitary renal masses need to bedifferentiated from renal cell carcinoma. We present a73-year-old man with a solitary renal mass treatedwith partial nephrectomy and histologic diagnosedwith a primary lymphoma of the kidney. In the preven-tive diagnosis, conventional radiology is unsuitable,contrast-enhanced renal ultrasound contributes to thediagnosis, and primary renal biopsy is useful.● We reviewed the medical literature and discuss pri-mary renal lymphoma, a rare disease with poor prog-nosis, whose treatment after postsurgical diagnosisbased on chemotherapy with R-CHOP (rituximab pluscyclophosphamide/doxorubicin/vincristine/prednisone).Clinical Genitourinary Cancer, Vol. 10, No. 1, 60-2 © 2012 Elsevier Inc. All rights reserved.Keywords: Imaging, Non-Hodgkin lymphoma, Partial nephrectomy, Primary renal lymphoma, Renal cell carcinomaCase Report60 Clinical Genitourinary Cancer March 20121558-7673/$ - see frontmatter © 2012 Elsevier Inc. All rights reserved.doi: 10.1016/j.clgc.2011.12.001Author's personal copyevaluate well the kidney because renal fluorine-18 fluorodeoxyglu-cose excretion interferes with kidney imaging.A preliminary characterization of lesion biopsy was not performeddue to the risk of seeding in this malignancy case. Due to the reducedrenal function (Glomerular filtration rate 59 mL/min), we decided toperform neprhon-sparing surgery enucleoresection. The patient wastreated with enucleoresection with renal warm ischemia for 22 min-utes. On histologic examination, macroscopic formation had a max-imum diameter of 6.8-cm, a thick fibrous wall that contained a largeamount of necrotic dampish material, yellowish-gray in color. Amicroscopic examination documented non-Hodgkin anaplasticlarge cell B extensively necrotic and capsule (Figure 1). The cells werepositive for CD 20 (Figure 2), PAX 5, Bcl 2, CD 30, vimen-tin, and CD 45, and were negative for CD3–, CKMNF116–,CK7–, CD10–, EMA–, CD56–, desmin–, -Myod1, Rod–, andCD31–.DiscussionPRL is defined as a NHL that starts in the parenchyma and does notshow invasion into adjacent lymph nodes. It is very rare and the diagno-sis of PRL is difficult.5 Lymphomas are malignant neoplasms character-ized by the proliferation of cells in the lymphoid tissue. Lymphomatousinvolvement of the genitourinary tract occurs in 3%-6% of cases and ismost common in the testis. The primary lymphoma of the kidney is aunique disease entity, because the kidney does not contain lymphatictissue. There is no evidence that the disease is the primary or initialmanifestation of systemic disease rapidly aggressive. It has been sug-gested that the lymphoma may originate from the renal sinus lymphaticsor lymph vessels located near the renal capsule and form cords that enterinto renal parenchyma, or follow chronic inflammation (such as chronicpyelonephritis, Sjogren’s Syndrome, Systemic Lupus Erythematosus,Epstein Barr Virus of mononucleosis).6 Many of the documented casesof renal lymphoma are secondary, and postmortem studies have foundthat approximately 50% of patients who died of NHL had renal involve-ment. PRL most commonly affects older male subjects, those older than60 years (mean age at diagnosis, 64 years), frequently unilateral, rarelybilateral.7,8 Large B-cell lymphoma is the most common histologic type,in up to approximately 30% of NHL, and is among the most clinicallyaggressive lymphoma. The clinical manifestations are similar to those ofother renal neoplasms also may present with: proteinuria, nephroticsyndrome, kidney failure. It is very aggressive, with rapid systemic dis-semination. Survival is extremely low, approximately 75% of patientsdie within the first year after surgery.6 The final prognosis can be im-proved by early diagnosis and systemic chemotherapy. There are severaloptions for the diagnostic evaluation of a possible involvement of thekidney, including renal ultrasonography, intravenous urography, CT,magnetic resonance imaging, and nuclear medicine.7 The ultrasoundcontrast agent is the mode of choice, whereas normal CT with contrasttechniques have not been a valuable diagnostic aid due to the extremevariability of findings that may be encountered in cases of renallymphoma.ConclusionsOverall, the prognosis of patients with renal lymphoma is less than1 year.5 The PRL is considered a systemic disease with renal mani-festation, even without evidence of extrarenal locations. The totalnephrectomy is not indicated, especially if it was possible to make apreoperative diagnosis.6 Patients with atypical findings suspected forrenal cell carcinoma should undergo a percutaneous renal biopsy,which has a sensitivity and a specificity of 70% -92% and 100%,respectively.5,6 Although renal lymphoma is often part of a general-ized process, our case appears to be limited to the kidney, so-calledstage IE diffuse large B-cell lymphoma. This early-stage aggressivelymphoma, may be highly curable with CHOP (cyclophosphamide,doxorubicin, vincristine [Oncovin], and prednisone] plus rituximab,based on the revised International Prognostic Index of Sehn et al,9but only 41%-53% of patients with advanced disease are alive anddisease free at a median follow-up of 3 years, especially when there isan overexpression of Bcl 2.5-9Figure 1 (A) Atrophic Renal Tubules. (B) Infiltration ofLymphoplasmacytic Cells. (C) Fibrous Capsule.(D) Tumor Cells. (E) Necrosis. Renal Lymphoma(Hematoxylin and Eosin)  30ABCDEFigure 2 Immunohistochemistry of Renal Lymphoma:coloration for CD20 Lymphocytes  400Clinical Genitourinary Cancer March 2012 61Author's personal copyDisclosureThe authors have stated that they have no conflicts of interest.References1. SEER Registry (surveillance epidemiology and end results). Available at: http://www.nci.nih.gov.Accessed: March 4, 2011.2. Omer HA, Hussein MR. Primary renal lymphoma. Nephrology (Carlton) 2007; 12:314-5.3. Stallone G, Infante B, Manno C, et al. Primary renal lymphoma does exist: case reportand review of the literature. J Nephrol 2000; 13:367-72.4. Besso L, Quercia AD, Daidola G, et al. Il coinvolgimento renale nei linfomi. G ItalNefrol 2010; 27(suppl 50):34-9.5. Pervez H, Shaikh M, Potti A, et al. Uncommon presentation of non-Hodgkin’slymphoma. Case 3. Primary renal lymphoma. J Clin Oncol 2003; 21:567-9.6. Cyriac S, Rejiv R, Shirley S, et al. Primary renal lymphoma mimicking renal cellcarcinoma. Indian J Urol 2010; 26:441-3.7. Pinggera GM, Peschel R, Buttazzoni A, et al. A possible case of primary renal lym-phoma: a case report. Cases J 2009; 2:6233 [Web site]. Available at: http://www.casesjournal.com/casesjournal/article/view/6233. Accessed: March 1, 2011.8. Vázquez AF, Sánchez RC, Vicente Prados FJ, et al. Primary renal lymphoma: reportof three new cases and literature review. Arch Esp Urol 2009; 62:461-5.9. Sehn LH, Berry B, Chhanabhai M, et al. The revised International PrognosticIndex (R-IPI) is a better predictor of outcome than the standard IPI for patientswith diffuse large B-cell lymphoma treated with R-CHOP. Blood 2007; 109:1857-61.Primary Lymphoma of the Kidney62 Clinical Genitourinary Cancer March 2012

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