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Year : 2006  |  Volume : 16  |  Issue : 4  |  Page : 901-904
Multilocular cystic nephroma of the kidney : A case report


Department Of Radiodiagnosis, Maulana Azad Medical College And Associated Lok Nayak Hospital, Jawahar Lal Nehru Marg, New Delhi - 110002, India

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Date of Submission30-May-2006
Date of Acceptance10-Oct-2006
 

Keywords: Renal tumour, benign, cystic nephroma

How to cite this article:
Singh S, Chowdhury V, Dixit R, Manchanda A. Multilocular cystic nephroma of the kidney : A case report. Indian J Radiol Imaging 2006;16:901-4

How to cite this URL:
Singh S, Chowdhury V, Dixit R, Manchanda A. Multilocular cystic nephroma of the kidney : A case report. Indian J Radiol Imaging [serial online] 2006 [cited 2019 Aug 24];16:901-4. Available from: http://www.ijri.org/text.asp?2006/16/4/901/32379

   Introduction Top


Multilocular cystic nephroma (MLCN) is an uncommon renal neoplasm that is characterized by a well circumscribed encapsulated mass that contains multiple non communicating fluid filled locules. The association of thin septa and large locules are suggestive findings for multilocular cystic nephroma [1]. The differential diagnosis includes a cystic Wilm's tumour in children and a cystic renal cell carcinoma in adults. Although new imaging techniques are available, surgical excision and histologic analysis of the tumour are the only effective methods to distinguish benign from malignant cystic lesions of the kidney. The need for proper diagnosis and designation of these tumours is highlighted in this case as these tumours are benign and carry an excellent prognosis [2].


   Case report Top


A 6 months old female presented with a palpable mass in her left upper abdomen. There were no other complaints. The routine hemogram was normal. CXR of the patient was normal. Ultrasound examination of the abdomen showed a multiseptate cystic mass of the left kidney. There were multiple circumscribed sonolucent cysts that had no communication with each other or the renal pelvis and none of the cysts exceeded 3 cm in size. The intervening septae were thin [Figure - 1]. Colour Doppler showed no flow at the capsule and the septa [Figure - 2]. Computed tomography showed multiple hypodense round or oval cysts with thin intervening septa. No calcification, no haemorrhage or fluid debris level was seen. No contrast enhancement was seen in the cysts but minimal enhancement was seen in the septa. Enhancing compressed renal parenchyma was seen posterior to the mass [Figure - 3]. The cysts had a CT number slightly greater than that of water density [Figure - 4]. Multiplanar coronal CT reconstruction showed the cystic mass replacing almost the entire left kidney [Figure - 5]. Pre-operative diagnosis of multilocular cystic nephroma and a cystic Wilm's tumour were considered. The patient underwent a left nephrectomy. No adhesions were found between the tumour and the surrounding tissue. The cut section showed an encapsulated mass composed of multiple discrete cysts filled with clear serous fluid measuring 0.1 to 3 cm. There was no solid tissue. The cyst wall was thin translucent measuring less than 2 mm (Fig. 6). Microscopically the cysts were lined by columnar to flat epithelial cells. The intervening stroma was loose with fibroblast few tubules and inflammatory cells (Fig. 7) consistent with multilocular cystic nephroma. The patient was kept on follow up.


   Discussion Top


Multilocular cystic nephroma (MLCN) is a distinct renal tumour of uncertain origin. The tumour is bulky, well encapsulated, non infiltrating and is composed of multiple non communicating fluid filled locules. It is usually expansive and well circumscribed and generally replaces an entire pole of the involved kidney.

Powell et al in 1951 proposed a set of eight criteria for the diagnosis of multilocular cystic nephroma [3].These were(i)Unilateral involvement (ii) Solitary lesion (iii) multilocular (iv) non communication of the cysts with one another (v) non communication with the renal pelvis (vi) loculi lined by epithelium (vii) interlobular septa devoid of renal parenchyma (viii) residual renal tissue if present is normal. Two more criteria were added to distinguish multilocular cystic nephroma from cystic partially differentiated Wilm's tumour, namely the tissue in the cyst must be entirely differentiated tissue and there must not be any blastematous or embryonal element [4].

MLCNS occur from three months of age to adult life with rare occurrences in infants younger than three months of age. Presenting symptoms vary with the patient's age. In the child MLCNS are predominantly evaluated because of a painless abdominal mass while in an adult the most common presentations are abdominal pain and hematuria. Hematuria is often associated with herniation of the tumour into the renal pelvis. This results in attenuation of the thin layer of transitional cell epithelium that covers the pelvic mass and leads to tissue necrosis, ulceration and finally bleeding. Less frequent findings include hypertension and urinary tract infection.

On plain radiography a soft tissue haze is seen with displacement of bowel loops. Calcification is uncommon but may be seen in older patients as curvilinear and in peripheric location [5]. On excretory urogram, kidneys function normally with MLCN.

Ultrasound is an excellent tool and findings relate to the amount of stroma and the size of the locules. When the cysts are large enough the US will demonstrate a cluster of echo free fluid filled locules separated by connective tissue and this pattern is suggestive of MLCN. Occasionally, however when the locules are small, the sonogram will demonstrate a nonspecific, complex intrarenal mass [6].

With colour Doppler US, it is possible to evaluate lesion vascularity non invasively.

As in US, CT findings correlate with cyst size and amount of stromal tissue. In most cases a large multiloculated mass is identified. The septa enhance with intravenous contrast medium due to their vascularity. The locules have CT numbers that are slightly greater than the CT number of water and they do enhance or accumulate contrast.

On angiographic examination, the MLCN is generally hypovascular but may be avascular or hypervascular. Thus angiographic features are not specific for differentiated diagnosis and should be performed only if outlining the vascular anatomy is important in surgical planning.

Multicystic dysplastic kidney, cystic partially differentiated nephroblastoma, malignant necrotic and haemorrhagic masses (renal cell carcinoma), Wilm's tumour and cystic mesoblastic nephroma should be included in the differential diagnosis [7]. Multicystic dysplastic kidney shows no function on excretory urogram and is characterized by multiple non communicating cysts of varying sizes and is a condition generally detected in the newborn period and antenatally. The differential diagnosis with cystic partially differentiated nephroblastoma is not possible based on imaging findings alone. The only differentiation between these two lesions is histological [4]. Cystic partially differentiated nephroblastoma contains blastema [5].

Wilm's tumour is the most common renal neoplasm in childhood (mean age of presentation 3 yrs) and shows distortion of the pelvicalyceal system on excretory urogram. Cystic necrosis is seen on US and CT in less than 10% cases. Variable patterns of calcification may be seen. Renal cell carcinoma is rare in children and does not pose a problem in that age group. When it occurs, haemorrhage and necrotic degeneration in these tumours may mimic MLCN.

Mesoblastic nephroma commonly presents in the first 3 months of life. The usual sonographic appearance is that of a huge solid echogenic mass with central hypoechoic areas caused by haemorrhage or necrosis. It is not encapsulated and may penetrate the renal capsule extending into the perinephric tissue. CT demonstrates a heterogeneous mass.

Although new imaging techniques are available, surgical intervention and histology is the only effective method to differentiate multilocular renal cyst from a malignant lesion of the kidney. Since carcinomatous degeneration may occur within the wall of such tumours, especially in Von Hippel Lindau disease, surgery is the treatment of choice for multilocular cystic nephroma [1]. The extent of surgery reported in literature has ranged from complete excision, partial nephrectomy to radical nephrectomy [8].

Thus multilocular cystic nephroma is an unique and characteristic renal lesion with benign biological behaviour. Because cystic Wilm's tumour and cystic renal cell carcinoma are difficult to distinguish from multilocular cystic nephroma on the basis of imaging findings alone. Surgical intervention is the only effective method to differentiate them and is the treatment of choice. Although no evidence of local recurrence or metastatic disease has been reported in literature a long term follow up is encouraged [7].

 
   References Top

1.Ley P, Helenon O, Merraus S, Paraf P et al. Cystic tumours of the kidney in adults : Radio-Histopathologic correlations. J. Radiol 1999; 80 (2): 121-133.  Back to cited text no. 1    
2.Sharma MC, Kashyap S, Kataria R, Gupta A. Spectrum of cystic variants of Wilm's tumour. Indian J Pathol Microbiol 1999; 42 (1): 107-111.  Back to cited text no. 2    
3.Powell T, Shackman R, Johnson HD. Multilocular cysts of the kidney. Br J Urol 1951; 23: 142.  Back to cited text no. 3  [PUBMED]  
4.Joshi VV, Beckwith JB. Multilocular cyst of the kidney (cystic nephroma) and cystic partially differentiated nephroblastoma : terminology and criteria for diagnosis. Cancer 1989; 64: 466-79.  Back to cited text no. 4  [PUBMED]  
5.Agrons GA, Wagner BJ, Davidson AJ, Suarez ES. Multilocular cystic renal tumour in children radiologic - pathologic correlation. Radiographics 1995; 15: 653-669.  Back to cited text no. 5  [PUBMED]  
6.Banner MP, Pollack HM, Chalten J, Witzleben C. Multilocular renal cysts : radiologic - pathologic correlation. AJR 1981; 36: 239-247.  Back to cited text no. 6    
7.Castillo O, Boyle E, Kramer S. Multilocular cysts of kidney. A study of 29 patients and review of literature. Urology 1991; 37(2): 156-162.  Back to cited text no. 7    
8.Sacher P, Willi UV, Niggli F, Stallmach T. Cystic nephroma : a rare benign tumour. Paed Surg Int 1998; 13: 197-199.  Back to cited text no. 8    

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Correspondence Address:
S Singh
212, Sfs Flats, Phase Iv, Ashok Vihar, Delhi - 110052
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0971-3026.32379

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    Figures

  [Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4], [Figure - 5], [Figure - 6], [Figure - 7]



 

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