Introduction
Background
Lymphoma of the kidneys is diagnosed at the time of autopsy in almost one half of cases. It is rarely suspected on conventional urologic studies, such as intravenous urography. However, renal involvement with lymphoma is commonly discovered in patients with known lymphoma, who may be diagnosed, staged, or followed by cross-sectional imaging, such as computed tomography (CT) scanning or magnetic resonance imaging (MRI).1
Pathophysiology
Familiarity with tumor growth and the mechanism of lymphoma spread are crucial to understanding the resultant imaging patterns of organ involvement. Renal lymphoma is often a secondary process that is discovered in patients with known lymphoma.
Contrast-enhanced computed tomography (CT) scan obtained during the pyelographic phase for staging in a 17-year-old male adolescent with known lymphoma. This image reveals a hypoattenuating nodular pattern of different-sized lesions in both kidneys that represent bilateral renal lymphoma. Hypoattenuating nodes are also shown in the retroperitoneum.
Computed tomography scan in a 17-year-old male adolescent with known lymphoma (same patient as in Image above). This image was obtained 7 months after the initiation of chemotherapy in the patient and reveals complete resolution of the bilateral hypoattenuating lesions. The appearance and function of the kidneys are normal. Note the complete resolution of the retroperitoneal nodes.
Hematogenous involvement of the kidneys usually results in a bilateral distribution of the tumor foci within the renal cortex. Tumor proliferation begins in the interstitium and the underlying nephrons; however, collecting systems and blood vessels provide a framework for tumor growth. Infiltrative growth results in preservation of the parenchymal structures and renal contour; consequently, detection is often difficult, and renal involvement can be easily missed. As lymphomatous tumors enlarge, the surrounding renal parenchyma is compressed and destroyed, and continuous tumor infiltration results in the formation of expansile renal masses. Nonuniform growth can result in single or conglomerate masses that extend beyond the renal contour and displace the collecting system; thus, when there is a single mass, it can resemble primary renal neoplasms.
Buyukpamukçu et al studied 104 pediatric patients with renal involvement from non-Hodgkin lymphoma.2 A renal infiltration pattern was reported to be nodular in 62 children (59.6%) and diffuse in 40 others (38.5%). Two patients had tumoral masses of renal origin (1.9%). Seventy-five children had bilateral renal involvement (72.1%), and 29 had unilateral involvement. The overall survival rate was 42.5% (median follow-up of 64 mo).
The mechanism of development of primary renal lymphoma is unclear, and this condition as a clinical entity has been disputed because the kidneys reportedly do not contain lymphatic tissue.3,4,5 Most of the few reported cases of primary renal lymphoma showed rapid systemic progression and a poor prognosis. Nevertheless, primary lymphoma of the kidneys represents a rare entity which must be considered in cases of unusual renal masses or otherwise unexplained renal symptoms.4
Frequency
International
The incidence of renal involvement in patients with lymphoma has been reported to be 34-62% in several autopsy case series. However, there is no agreement whether or not renal involvement is an initial manifestation of a rapidly systemic disease. In addition, most of the reported cases are of questionable validity because of incomplete staging and the lack of autopsy studies.5
Renal involvement occurs more commonly in non-Hodgkin lymphoma than in Hodgkin lymphoma. The incidence rate is often underestimated in imaging studies, with CT scanning depicting renal involvement in up to 8% of all patients undergoing routine CT scanning for the staging of lymphoma. In part, this discrepancy reflects the fact that patients with presumed lymphomatous renal involvement rarely undergo nephrectomy or biopsy. The disease process is silent and often poorly documented.
Mortality/Morbidity
- Because involvement of the kidneys usually indicates disseminated disease, in the absence of proper clinical treatment, the prognosis is poor. The disease may present with progressive renal failure of either the oliguric or nonoliguric type. In primary renal lymphoma, survival is extremely poor: 75% of patients die in less than 1 year. The prognosis may be improved by early detection of the disease and by performing systemic chemotherapy.
- With appropriate treatment, renal lesions may completely regress, often with minimal scarring within the renal parenchyma (see Images below and Images 1-2 in Multimedia).
Contrast-enhanced computed tomography (CT) scan obtained during the pyelographic phase for staging in a 17-year-old male adolescent with known lymphoma. This image reveals a hypoattenuating nodular pattern of different-sized lesions in both kidneys that represent bilateral renal lymphoma. Hypoattenuating nodes are also shown in the retroperitoneum.
Computed tomography scan in a 17-year-old male adolescent with known lymphoma (same patient as in Image above). This image was obtained 7 months after the initiation of chemotherapy in the patient and reveals complete resolution of the bilateral hypoattenuating lesions. The appearance and function of the kidneys are normal. Note the complete resolution of the retroperitoneal nodes.
Race
Renal lymphoma demonstrates no racial predilection.
Sex
Renal lymphoma demonstrates no sex predilection. However, it is reportedly seen more often in male patients.
Age
Renal lymphoma occurs in all age groups. The disease usually affects adults (average age: 60 y); however, renal lymphoma has also been reported in childhood.
Anatomy
The kidneys are bean-shaped structures that are located in the retroperitoneal region and surrounded by the Gerota fascia. The renal parenchyma is enclosed in a thin, fibrous, glistening membrane that represents a true capsule. The lateral edge of the kidneys is convex, whereas the medial border is concave, with a marked depression or notch, termed the hilus. In adults, each kidney weighs 120-170 g and is approximately 11 cm long, 2.5 cm thick, and 5 cm wide.
Usually, the left kidney is approximately 1.5 cm higher than the right kidney. When the patient is lying in the supine position, the superior pole of the left kidney is at the level of the 12th thoracic vertebra, and the inferior pole is at the level of the 3rd lumbar vertebra. With a patient in the erect position or during deep inspiration, both kidneys may descend as low as near the iliac crest or even lower.
As a result of the proximity of the kidneys to the psoas muscles, the oblique course of these muscles causes slight lateral displacement of the lower pole of each kidney. Between the 2 kidneys are the aorta and inferior vena cava, as well as the celiac plexus and the ganglia of the autonomic nervous system. The renal artery and vein anterior to the renal pelvis run medially and anteriorly from the hilus of each kidney. The kidneys are usually supplied by one renal artery each; however, it is not uncommon to see 2 or more renal arteries originate from the aorta. Although the left renal vein normally courses anterior to the aorta to drain into the vena cava, this vein can occasionally run behind the aorta (retroaortic renal vein) or be anterior and posterior (circumaortic) to the aorta.
Presentation
Patients with renal lymphoma may not be symptomatic; however, renal function laboratory studies occasionally reveal elevated blood urea nitrogen and creatinine levels, which may suggest an obstruction. Therefore, the patient may need to undergo radiologic evaluation.6,7,8,9,10,11
Sometimes, patients present with nonspecific signs and symptoms, including flank pain, weight loss, hematuria, or a palpable mass. Occasionally, symptoms of back pain are attributed to the patient's known underlying lymphoma. The nature of lymphomatous infiltration around the renal pedicle is such that it may not cause vascular compromise; however, the tumor usually surrounds the renal vascular pedicle, and the tumor mass can obstruct urine flow, resulting in hydronephrosis.
Preferred Examination
Numerous diagnostic studies are available for the evaluation of renal involvement from lymphoma, including intravenous urography, ultrasonography, nuclear medicine, MRI, and CT scanning. Although each technique may provide useful information in the detection, characterization, and staging of the disease, CT scanning remains the most sensitive, efficient, and comprehensive modality.12,13
Urography provides information regarding involvement of the collecting system, the functional status of the kidneys, and the severity of hydronephrosis. Ultrasonography is a good screening examination, particularly for the assessment and follow-up of patients with hydronephrosis. In comparison, nuclear medicine studies, particularly those that use gallium-67 citrate, have a higher degree of specificity in the detection of lymphomatous infiltration of the kidneys. MRI provides information for characterizing the lesion, and it is also helpful in assessing the renal vascular pedicle.
Contrast-enhanced computed tomography scan in a 15-year-old male adolescent with non-Hodgkin lymphoma. This image shows a bilateral pattern of multiple small nodules that are almost of equal size and involve both kidneys.
Sagittal baseline sonogram in a 15-year-old male adolescent with non-Hodgkin lymphoma (same patient as in Image 3 in Multimedia). This image shows near-normal architecture of the left kidney. Both kidneys were reported as normal (left kidney size, 11.4 X 5.7 cm), and no indication of hydronephrosis was noted.
Contrast-enhanced computed tomography scan obtained during the nephrographic phase in a 93-year-old woman with non-Hodgkin lymphoma, in whom renal lymphoma mimicked renal cell carcinoma. This image reveals a single mass with heterogeneous attenuation that involves the right kidney, as well as an associated lymphadenopathy in the right side of the retroperitoneum. The findings suggest renal cell carcinoma with ipsilateral adenopathy. A normal variant of an extrarenal pelvis is noted in the left side.
Contrast-enhanced, breath-hold, T1-weighted magnetic resonance image (MRI) (repetition time, 190 ms; echo time, 4 ms) in a 93-year-old woman with non-Hodgkin lymphoma (same patient as in Image 5 in Multimedia). This image shows a mass with heterogeneous signal intensity in the right kidney; areas of low signal intensity reveal necrosis in the mass. MRI was performed to assess the renal vascular pedicle and inferior vena cava, both of which were free of tumor. Because of the patient's history of non-Hodgkin lymphoma (for which she received treatment), biopsy was performed to exclude renal cell carcinoma. The diagnosis of renal lymphoma was proven, and the patient received appropriate treatment.
CT scanning is the diagnostic modality of choice in patients with suspected renal masses (see Images 1-6). CT scans depict renal involvement in most patients with lymphoma, often help in defining the extent of disease within and outside of the kidneys, and provide information for staging of the patient's underlying malignancy. In addition, the result of treatment can be assessed on follow-up CT scans. The CT scan examination must be done with intravenous contrast media. Therefore, if contrast media cannot be used in patients for reasons such as renal failure or allergy to iodine, MRI then becomes the diagnostic modality of choice.
Limitations of Techniques
Intravenous urography can depict only the renal collecting system and suggestive large masses that have caused calyceal distortion. Small parenchymal masses and tumors around the kidney and in the retroperitoneum are often not demonstrated with this diagnostic examination.
On sonograms, the parenchymal masses are often hypoechoic, reflecting tissue homogeneity, and they can be misidentified as renal cysts.
Regarding nuclear medicine studies, although gallium-67 citrate is an isotope that often accumulates in lymphomatous tissue, it can also be taken up by inflammatory masses.
Nonenhanced CT scan studies or MRIs can cause masses to be missed, particularly when such masses are small.
Patient Education: For excellent patient education resources, visit eMedicine's Cancer and Tumors Center. Also, see eMedicine's patient education article Renal Cell Cancer.
Differential Diagnoses
Angiomyolipoma, Kidney
Metastatic Cancer, Unknown Primary Site
Renal Cell Carcinoma
Other Problems to Be Considered
Renal metastasis
Renal abscess
More on Kidney, Lymphoma |
 Overview: Kidney, Lymphoma |
| Imaging: Kidney, Lymphoma |
| Follow-up: Kidney, Lymphoma |
| Multimedia: Kidney, Lymphoma |
| References |
| Further Reading |
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References
Ladha A, Haider G. Primary renal lymphoma. J Coll Physicians Surg Pak. Sep 2008;18(9):584-5. [Medline].
Buyukpamukçu M, Varan A, Aydin B, et al. Renal involvement of non-Hodgkin's lymphoma and its prognostic effect in childhood. Nephron Clin Pract. 2005;100(3):c86-91. [Medline].
Tefekli A, Baykal M, Binbay M, Barut M, Muslumanoglu AY. Lymphoma of the kidney: primary or initial manifestation of rapidly progressive systemic disease?. Int Urol Nephrol. 2006;38(3-4):775-8. [Medline].
Gellrich J, Hakenberg OW, Naumann R, et al. Primary renal non-Hodgkin's lymphoma - a difficult differential diagnosis. Onkologie. Jun 2002;25(3):273-7. [Medline].
Stallone G, Infante B, Manno C, et al. Primary renal lymphoma does exist: case report and review of the literature. J Nephrol. Sep-Oct 2000;13(5):367-72. [Medline]. [Full Text].
Amirzargar MA, Dadras F, Khoshjoo F, et al. Non-Hodgkin's lymphoma in autosomal dominant polycystic kidney disease, 12 years after renal transplantation. Saudi J Kidney Dis Transpl. Jul-Sep 2007;18(3):419-21. [Medline].
Becker AM, Bowers DC, Margraf LR, Emmons J, Baum M. Primary renal lymphoma presenting with hypertension. Pediatr Blood Cancer. Jun 15 2007;48(7):711-3. [Medline].
Silverman SG, Mortele KJ, Tuncali K, Jinzaki M, Cibas ES. Hyperattenuating renal masses: etiologies, pathogenesis, and imaging evaluation. Radiographics. Jul-Aug 2007;27(4):1131-43. [Medline].
Jindal B, Agarwala S, Bakhshi S, Jain V, Gupta AK, Kumar R, et al. Bilateral primary renal lymphoma with orbital metastasis in a child. Pediatr Blood Cancer. Dec 17 2008;[Medline].
Kato Y, Hasegawa M, Numasato S, Monma N, Fujioka T. Primary mucosa-associated lymphoid tissue-type lymphoma arising in the kidney. Int J Urol. Jan 2008;15(1):90-2. [Medline].
Fang FS, Zhu HL, Song ZG, Lu XC. [Three cases of primary renal lymphoma]. Zhongguo Shi Yan Xue Ye Xue Za Zhi. Oct 2007;15(5):1107-11. [Medline].
Hunter S, Samir A, Eisner B, et al. Diagnosis of renal lymphoma by percutaneous image guided biopsy: experience with 11 cases. J Urol. Nov 2006;176(5):1952-6; discussion 1956. [Medline].
Sheth S, Ali S, Fishman E. Imaging of renal lymphoma: patterns of disease with pathologic correlation. Radiographics. Jul-Aug 2006;26(4):1151-68. [Medline]. [Full Text].
El-Sharkawy MS, Siddiqui N, Aleem A, Diab AA. Renal involvement in lymphoma: prevalence and various patterns of involvement on abdominal CT. Int Urol Nephrol. Jun 5 2007;epub ahead of print. [Medline].
Murphy JJ, Tawfeeq M, Chang B, Nadel H. Early experience with PET/CT scan in the evaluation of pediatric abdominal neoplasms. J Pediatr Surg. Dec 2008;43(12):2186-92. [Medline].
Ravinsky E, Morales C. Diagnosis of lymphoma by image-guided needle biopsies: fine needle aspiration biopsy, core biopsy or both?. Acta Cytol. Jan-Feb 2005;49(1):51-7. [Medline].
Castellucci P, Zinzani P, Pourdehnad M, et al. 18F-FDG PET in malignant lymphoma: significance of positive findings. Eur J Nucl Med Mol Imaging. Jul 2005;32(7):749-56. [Medline].
Cohan RH, Dunnick NR, Leder RA, Baker ME. Computed tomography of renal lymphoma. J Comput Assist Tomogr. Nov-Dec 1990;14(6):933-8. [Medline].
Colovic M, Hadzi-Djokic J, Cemerikic V, et al. Primary MALT lymphoma of the kidney. Hematol Cell Ther. Nov 1999;41(5):229-32. [Medline].
Honda H, Barloon TJ, Franken EA Jr, Garneau RA, Smith JL. Clinical and radiologic features of malignant neoplasms in organ transplant recipients: cyclosporine-treated vs untreated patients. AJR Am J Roentgenol. Feb 1990;154(2):271-4. [Medline]. [Full Text].
Iselin CE, Leder RA, Lacey J, Gockerman JP, Paulson DF. Renal lymphoma in an azotemic patient--usefulness of magnetic resonance imaging. Scand J Urol Nephrol. Apr 1999;33(2):129-30. [Medline].
Jafri SZ, Bree RL, Amendola MA, et al. CT of renal and perirenal non-Hodgkin lymphoma. AJR Am J Roentgenol. Jun 1982;138(6):1101-5. [Medline]. [Full Text].
Lorigan JG, David CL, Shirkhoda A, Eftekhari F, Alexanian R. Macroglobulinaemic lymphoma presenting with perirenal masses. Br J Radiol. Nov 1988;61(731):1077-8. [Medline].
Murthy S, Sharkey RM, Goldenberg DM, et al. Lymphoma imaging with a new technetium-99m labelled antibody, LL2. Eur J Nucl Med. 1992;19(6):394-401. [Medline].
Parkhurst JB, Foster P, Johnson SF, Leonard JC. Upstaging of non-Hodgkin's lymphoma in a child based on 67Gallium scintigraphy. J Pediatr Hematol Oncol. Mar-Apr 1998;20(2):174-6. [Medline].
Porcaro AB, D'Amico A, Novella G, et al. Primary lymphoma of the kidney. Report of a case and update of the literature. Arch Ital Urol Androl. Mar 2002;74(1):44-7. [Medline].
Richards MA, Mootoosamy I, Reznek RH, Webb JA, Lister TA. Renal involvement in patients with non-Hodgkin's lymphoma: clinical and pathological features in 23 cases. Hematol Oncol. Mar-Apr 1990;8(2):105-10. [Medline].
Salem Y, Pagliaro LC, Manyak MJ. Primary small noncleaved cell lymphoma of kidney. Urology. Sep 1993;42(3):331-5. [Medline].
Semelka RC, Kelekis NL, Burdeny DA, et al. Renal lymphoma: demonstration by MR imaging. AJR Am J Roentgenol. Apr 1996;166(4):823-7. [Medline]. [Full Text].
Sheeran SR, Sussman SK. Renal lymphoma: spectrum of CT findings and potential mimics. AJR Am J Roentgenol. Oct 1998;171(4):1067-72. [Medline]. [Full Text].
Shirkhoda A, Staab EV, Mittelstaedt CA. Renal lymphoma imaged by ultrasound and gallium-67. Radiology. Oct 1980;137(1 Pt 1):175-80. [Medline]. [Full Text].
Smith PA, Marshall FF, Fishman EK. Spiral computed tomography evaluation of the kidneys: state of the art. Urology. Jan 1998;51(1):3-11. [Medline].
Truong LD, Caraway N, Ngo T, et al. Renal lymphoma. The diagnostic and therapeutic roles of fine-needle aspiration. Am J Clin Pathol. Jan 2001;115(1):18-31. [Medline]. [Full Text].
Tsang K, Kneafsey P, Gill MJ. Primary lymphoma of the kidney in the acquired immunodeficiency syndrome. Arch Pathol Lab Med. May 1993;117(5):541-3. [Medline].
Further Reading
Related eMedicine topics
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Renal Cell Carcinoma
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Clear Cell Sarcoma of the Kidney
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Clinical trials
Carboxyamidotriazole and Paclitaxel in Treating Patients with Advanced Solid Tumors or Refractory Lymphomas
Total-Body Irradiation and Chemotherapy Followed by Peripheral Stem Cell Transplantation in Treating Patients with Hematologic Cancer or Kidney Cancer
Clinical guidelines
ACR Appropriateness Criteria® follow-up of renal cell carcinoma. American College of Radiology - Medical Specialty Society. 1996 (revised 2007). 5 pages. NGC:005994
ACR Appropriateness Criteria® renal cell carcinoma staging. American College of Radiology - Medical Specialty Society. 1995 (revised 2007). 8 pages. NGC:006001
The role of cytoreductive nephrectomy in metastatic renal cell cancer: a clinical practice guideline. Program in Evidence-based Care - State/Local Government Agency [Non-U.S.]. 2006 Apr 10. 19 pages. NGC:004955
Interleukin-2 in the treatment of patients with unresectable or metastatic renal cell cancer: a clinical practice guideline. Program in Evidence-based Care - State/Local Government Agency [Non-U.S.]. 2006 Jun 8. 27 pages. NGC:005223
Keywords
renal lymphoma, kidney lymphoma, kidney cancer, renal lymphoma, renal cancer, lymphoma of the kidney, metastatic involvement of the kidneys by lymphoma, perirenal halo sign, renal mass
