|Year : 2016 | Volume
| Issue : 4 | Page : 130-132
Hira Lal, Vivek Agarwal, Suprava Naik
Department of Radiology, SGPGIMS, Lucknow, Uttar Pradesh, India
|Date of Web Publication||7-Nov-2016|
Department of Radiology, SGPGIMS, Lucknow, Uttar Pradesh
Source of Support: None, Conflict of Interest: None
Renal lymphangiomas are rare benign mass-like renal conditions that refer to the presence of multiple cysts in both the renal sinus and the renal parenchyma. It has been found in both adults and children. The etiology is unknown, but it has been thought to be the result of lymphatic obstruction. Renin-dependent hypertension may be the presenting clinical complaint. The purpose of this study is to describe the radiological appearances of a rare case of renal lymphangioma that we encountered in our hospital.
Keywords: Benign, lymphangioma, mass
|How to cite this article:|
Lal H, Agarwal V, Naik S. Renal Lymphangioma. J Integr Nephrol Androl 2016;3:130-2
| Introduction|| |
Lymphangioma is a benign vascular lesion with lymphatic differentiation. Although most are found in the neck and axillae, they may also arise in the retroperitoneum where they are seen as thin-walled multicystic masses. Renal lymphangioma is also referred to as renal lymphangiectasia or lymphangiomatosis. It is seen most frequently in the renal sinus or perinephric space as a multiloculated cystic mass.  Rather than a neoplasm, it is thought to be a developmental anomaly of the lymphatic system and is often bilateral.  Most patients are asymptomatic, and treatment is seldom needed. We report a case of a large renal lymphangioma arising from the lower pole of right kidney and also discuss the radiological appearances of this rare benign condition.
| Case report|| |
A 22-year-old female patient presented in the outpatient department with complaints of pain in the right lumbar region for last few months. There was no history of burning micturition, hematuria, fever, or history of the previous urinary stone disease/urinary tract infection. The pain was nonradiating, mild, dull aching in nature. The patient was normotensive. Biochemical investigations and urinalysis showed clear urine, yellowish colored, pH - 5, glucose - 80 mg/dL, white blood cells - ≤2 WBCs/hpf, protein - 60 mg/dL, and no red blood cells. Renal and liver function parameters were normal. Chest X-ray was within normal limits. After a thorough examination, the patient was sent to radiology for ultrasound evaluation of the lumbar pain.
The patient was examined by an experienced ultrasonologist for urinary tract evaluation. Ultrasound showed a large relatively well defined hypoechoic multicystic lesion at the lower pole of right kidney. Distal acoustic enhancement was present, revealing multicystic nature. No intralesional color flow was noted on Doppler study [Figure 1]. Similar small lesions were also seen at lower pole of left kidney. Computed tomography (CT) (Brilliance, Philips 64), with and without intravenous contrast, revealed a large hypodense nonenhancing mass lesion at lower pole of right kidney. Similar lesions were seen at lower pole of the left kidney. No calcifications or evidence of intralesional bleed was seen. Calyceal system at lower pole of the right kidney appeared dilated [Figure 2].
|Figure 1: (a) A large relatively well-defined hypoechoec multicystic lesion at mid-lower pole of right kidney (yellow arrow). Dilated intralesional pelvicalyceal system is noted (green arrow). Normal upper pole renal parenchyma is seen (red arrow). (b) Color Doppler study shows no flow in the lesion. Normal renal parenchyma at upper pole shows color flow|
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|Figure 2: (a) Precontrast axial image shows a large hypodense mass lesion at mid-lower pole of right kidney (green arrow). No calcifications or evidence of intralesional bleed is seen. Normal renal parenchyma is seen (red arrow). (b) Postcontrast coronal image showing no nodular enhancement within the lesion (green arrow). Normal renal parenchyma at upper pole shows normal enhancement (red arrow). Similar lesions are seen at lower pole of left kidney (yellow arrow)|
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Subsequently, magnetic resonance imaging (MRI) (Signa, General Electric Medical Systems, Milwaukee, USA) was done to further characterize the lesion. The parameters for these MRI examinations were as follows: axial T1-weighted conventional spin-echo images (TR/TE, 1360/7.2); coronal nonfat-suppressed T2-weighted fast spin-echo images (TR/TE, 5400/111), and fat-suppressed T2-weighted spin-echo images (TR/TE; 2820/111); axial and coronal postcontrast fat suppressed T1 (TR/TE; 1000/7.3).
MRI showed a large reniform septate mass with heterogeneous low signal intensity on T1-weighted images and with high signal intensity on T2-weighted images at lower pole of the right kidney. Right-sided renal vessels were displaced anteriorly by the lesion. Pelvicalyceal system at lower pole appeared dilated. No postcontrast enhancement was noted [Figure 3] and [Figure 4].
|Figure 3: (a) Coronal T2-weighted image shows a high signal intensity lesion at mid-lower pole of right kidney. Hypointense internal septae are also seen (yellow arrow). Pelvi-calyceal system at lower pole appeared dilated. Similar small lesions are seen at lower pole of the left kidney (red arrow). (b) Axial T2-weighted image shows the hyperintense mass lesion with hypointense internal septae (yellow arrow)|
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|Figure 4: (a) Axial precontrast T1-weighted image shows a low signal intensity lesion at mid-lower pole of the right kidney (red arrow). (b) Axial postcontrast T1-weighted image shows minimally enhancing internal septae. No solid nodular enhancement is seen|
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The patient is being managed on conservative treatment as patient refused for surgery.
| Discussion|| |
Renal lymphangiomatosis is a rare disorder in which lymphatic tissue fails to develop a normal communication with the rest of the lymphatic system. Cystic masses develop, usually in the perinephric space immediately adjacent to the kidney as a result of obstruction of larger lymphatics that drain through the renal pelvis. Our case was unique in that the lesion was completely replacing mid and lower pole of the kidney without dilatation of perirenal lymphatics. There is no sex predilection and the tumor mostly affects adults. Association with monosomy X, trisomy 7q, and defects in the von Hippel-Lindau gene has been reported. Histologically, these lesions contain lymphatic/proteinaceous fluid and are lined by an endothelial layer. 
Abnormal lymphatic channels may dilate to form a cystic mass that may be unilocular or multilocular; macrocystic, or microcystic. The size and location of this disease are predominantly dependent on the site of the lymphatic obstruction. If the larger lymphatic vessels in the renal pedicle are involved, diffuse renal lymphangiectasia, i.e., lymphangiomatosis, can develop.  In contrast, if small intra-renal lymphatic vessels are involved, a localized renal mass may occur as in our case. Therefore, radiologic features of this disease can be variable from a small, localized mass to a larger, multiloculated cystic mass, although most cases are seen as the latter.
Patients may present with a palpable abdominal mass, renal colic, or perinephric cystic masses that may be found incidentally on cross-sectional imaging studies named as lymphangiomatosis.
Some patients may present with hematuria, hypertension, or chyluria.  Renal function is usually normal. Pregnancy may exacerbate the condition, leading to "decompensated lymphangiectasia." 
Depending on the size and location of lymphatic obstruction, radiologic features van vary variable from a small, localized unilocular mass to a larger, multiloculated cystic mass. On imaging, a lymphangioma presents as a uni- or multi-locular cystic lesion, focal, or diffuse. Contrast-enhanced imaging can sometimes show peripheral or septal enhancement. The lesion is usually T2 hyperintense and T1 hypointense at MRI. MRI signal intensity can vary if hemorrhage or debris is present in the lesion.  The perirenal space can be affected, and in the case of perirenal lymphangiomatosis, there are multiple cystic masses present bilaterally in the perirenal mesenchyme. Sometimes in association with a renal lymphangioma, linear fluid filled structures are noted in the retroperitoneum, which are probably dilated lymphatic channels.
Renal lymphangiomatosis must be distinguished from autosomal-dominant polycystic kidney disease (ADPKD).  Patients with ADPKD have innumerable parenchymal cysts that are usually bilateral. Microcystic lymphangiomas may appear solid on imaging and need to be differentiated from small renal cell carcinomas. However, renal cell carcinomas show significant solid arterial postcontrast enhancement. Other differentials include multilocular cystic renal cell carcinoma, mesoblastic nephroma, and localized cystic disease of the kidney.  Multilocular cystic renal cell carcinoma may show solid enhancing nodule or thick enhancing septae. Localized cystic disease of the kidney is usually unilateral with relatively larger cysts. Fine needle aspiration and cytological evaluation may be helpful in difficult cases.
Evolution of this lesion is variable, spontaneous resolution has been reported. Hence, generally no treatment is required in asymptomatic patients. Other cases may require percutaneous or laparoscopic aspiration, marsupialization of larger cysts, and nephrectomy. Sclerotherapy may be helpful in unilocular lesions. 
| Conclusion|| |
Localized renal lymphangioma is a rare condition and may present a diagnostic challenge especially for the inexperienced radiologists. Careful assessment using ultrasound or other cross-sectional modalities like CT/MRI may reveal the multiloculated nature of the lesion.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]