|Year : 2013 | Volume
| Issue : 1 | Page : 84-85
Adult benign multicystic nephroma: A diagnostic dilemma
Chandan Chatterjee, Dawood Khan, Utpal De
Department of General Surgery, Medical College and Hospital, Kolkata, West Bengal, India
|Date of Web Publication||14-Mar-2013|
46 Dr. Jagabandhu Lane, Kolkata, West Bengal
Source of Support: None, Conflict of Interest: None
Cystic nephroma, also called multilocular cystic nephroma, is a relatively rare, non-genetic, benign, unilateral renal multicystic lesion. The non-specific clinical findings and the paucity of information from imaging techniques make the preoperative dilemma from othercystic renal neoplasia; thus nephrectomy seems to be the most preferable treatment. We report a case of cystic nephroma in forty years male patient presented with asymptomatic lump in abdomen.
Keywords: Cystic nephroma, multilocular cystic nephroma, polycystic nephroma
|How to cite this article:|
Chatterjee C, Khan D, De U. Adult benign multicystic nephroma: A diagnostic dilemma. Med J DY Patil Univ 2013;6:84-5
| Introduction|| |
Multilocular cystic nephroma (MLCN) is a rare, non-genetic, benign, unilateral, renal multicystic lesion; grouped along the mixed epithelial - stromal tumour of kidney.  There is no proved theory concerning its pathogenesis, thus its origin is thought to be eitherdysplastic,hamartomas, or neoplastic.  The usual presentation is benign clinical course with bimodal age distribution,asymptomatic abdominal mass with nonspecific symptoms as abdominal pain, hematuria and urinary tract infection. 
| Case Report|| |
A 40 year male presented with vague abdominal pain in the left side of the abdomen for last 15 days. The pain was insidious in onset,with no radiation, no aggravating or relieving factor. There was no history of hematuria, no symptoms of renal failure or similar illness in the family. There was no complaining of any swelling in the abdomen. On examination the patient was alert conscious, cooperative with pulse rate-86/min, blood pressure-134/80 mmHg with no evidence of anaemia, jaundice, clubbing, oedema, and cyanosis. Abdominal examinations revealed no definite mass in the abdomen. On deep palpation mild tenderness in the left lumber region was found. On investigation complete hemogram of the patient revealed Hb-11. 0 gm%, total leucocyte count-6900/cmm, total bilirubin-0.8 mg%, ALP-103IU, SGOT-34IU, SGPT-28IU. Ultrasonography of whole abdomen revealed left kidney is enlarged. A hypoechoiclesion measuring 8.9 × 3.9 cm size was seen at the lower pole of left kidney. No hydronephrosis or calculus was seen. Right kidney was normal. CT scan of whole abdomen revealed a variegated mass at lower pole of left kidney (45 × 39 mm) with an impression of renal cell carcinoma (RCC) [Figure 1].
|Figure 1: CT scan of abdomen showing a variegated mass in the lower pole of left kidney(45 × 39 mm)|
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A radical nephrectomy was planned and abdomen was opened by midline incision. Left kidney was explored. A growth was found at the lower pole. Pedicle of left kidney was ligated and cut. On gross examination of the left kidney there was amass present in the lower pole of the kidney approximately 10 cm × 5 cm × 3 cm in size. It was whitish, well circumscribed with a smooth surface, tumour extended to renal capsule without breaching it, renal vessels and ureters were normal. Post-operative period was uneventful. The patient was absolutely symptom free during follow up. Histopathology report showed features of benign multicysticnephroma [Figure 2] and IHC was positive for CK 19.
|Figure 2: Section showing the cystic locules lined by cuboidal orcolumnar epithelial cells. There is denudation of epithelium at places with collection of heamosederin in macrophage, lymphocyte andplasma cells. A prominent fibromuscularstroma with entrapped small tubules seen in the cyst wall|
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| Discussion|| |
The first case of cystic nephroma was reported in 1892 as cystic adenoma of the kidney.  MLCN is a rare renal tumour. In adults, the order of differential diagnosis should be as follows: benign multilocular cyst, mutilocular cystic RCC and a cystic RCC. MLCN is classified among the mixed epithelial stromal tumours though separate entity also exists.  Diagnostic criteria for MLCN include: unilateral involvement, solitary lesion,multilocular lesion, no communication of cyst wall with each other, locules lined by epithelium, intralocular septa devoid of renal parenchyma and no communication with the renal pelvis and normal residual tissue if present.  These criteria were partially revised by Joshi and Beckwith  in 1989. Our patient fulfils the above criteria.
Roentgenographic criteria include soft tissue mass with calcification.  Intravenous urography reveals well defined, intra renal mass in a normal functioning kidney. Rarely the kidney is non-visualized due to compression by pelvic herniation of the tumour. Sonography findings relate to size of the locules; either a non-specific complex intrarenal mass with multilocular configuration, discrete septa and sonolucent spaces. CT usually reveals a smooth multilocular mass and determines its perinephric extensions. Colour Doppler flow imaging shows avascular mass. MRI angiography is an alternative. At present there is no reliable clinical or radiographic means to differentiate cystic nephroma from RCC in adults. 
The non-specific clinical findings and the poor contribution of imaging examination render the exact preoperative distinction from other cystic renal neoplasm difficult. Thus taking into consideration the possible neoplastic nature and the difficulty in preoperative diagnosis, surgical excision of tumour is strongly advised for definite diagnosis and treatment.
| Conclusion|| |
The non-specific clinical findings and the poor contribution of imaging examination make the preoperative diagnostic dilemma from other cystic renal neoplasia; nephrectomy seems to be the most preferable treatment. Final diagnosis can be established in the Histopathology examination of the kidney specimen.
| Acknowledgment|| |
Dr. Utpal Kr Dutta, Principal, Medical College and Hospital.
| References|| |
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[Figure 1], [Figure 2]