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CASE REPORT
Year : 2002  |  Volume : 18  |  Issue : 2  |  Page : 150-151
 

Renal cell carcinoma in autosomal dominant polycystic kidney disease


Department of Urology, SGPGI, Lucknow, India

Correspondence Address:
Rakesh Kapoor
Department of Urology, SGPGIMS, Raebareli Road, Lucknow - 226 014
India
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Source of Support: None, Conflict of Interest: None


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Keywords: Renal Cell Carcinoma; Polycystic Kidney.


How to cite this article:
Raghavendran M, Kapoor R, Kumar A, Zaman W, Srivastava A, Mandhani A. Renal cell carcinoma in autosomal dominant polycystic kidney disease. Indian J Urol 2002;18:150-1

How to cite this URL:
Raghavendran M, Kapoor R, Kumar A, Zaman W, Srivastava A, Mandhani A. Renal cell carcinoma in autosomal dominant polycystic kidney disease. Indian J Urol [serial online] 2002 [cited 2019 Dec 14];18:150-1. Available from: http://www.indianjurol.com/text.asp?2002/18/2/150/37619



   Introduction Top


Walters and Braasch first reported association of ADPKD and RCC in 1934, and 30 such cases have been described in the literature. [1] Hereby we present one such case seen.


   Case Report Top


A 43-year-old male presented with left flank pain, hae­maturia and loss of appetite for last one month. He had a positive family history for ADPKD. Examination revealed a cachectic male, with bilateral renomegaly and left kid­ney harder than the right. Renal and liver function tests were normal. Renal ultrasound and CT scan showed pan­creatic cysts, bilateral renal cysts (multiple) and a large exophtic mass from left kidney with renal vein thrombus. CT scan confirmed the findings [Figure - 1],[Figure - 2]. Diagnostic aspirate cytology was positive for malignant cells. The patient was subjected to a radical nephrectomy. Histopa­thology revealed Grade IV RCC, RV thrombus and multi­ple cysts. He made uneventful recovery.


   Review Top


ADPKD is the most common cystic disease seen in 1: 500-1000 patients. [2] Risk of renal carcinoma in these pa­tients is controversial and reportedly <1%. [3] Coexistence of RCC and renal cyst in same kidney has been reported in only 1-2% of cases of RCC. Earlier differentiation between cyst and RCC was not apparent readily, so the cyst puncture, fluid aspiration and cytology were standard di­agnostic tests. But now these are needed less due to ad­vent of CT and MRI. Review of literature also revealed that earlier the majority of diagnoses were made after sur­gical exploration.

Keith et al concluded that ADPKD is not a risk factor for RCC, but in these patients RCC occurs at a younger age and there is increased incidence of bilateral, multi­centric, sarcomatoid tumours. [4] In addition to the above features, there may be an increased incidence of high grade tumours in these patients as seen in our case.

The next question is that of management and this is con­troversial. We preferred unilateral nephrectomy because of the following factors : Low incidence of RCC, morbidity of bilateral nephrectomy, management of anephric state and presence of no mass on contralateral exploration. However these patients need repeated imaging on regular follow­up. Multiple imaging modalities must be used. The devel­opment of small suspicious lesion in the remaining kidney necessitates aggressive workup including guided biopsies and if need be open exploration.

 
   References Top

1.Walters W, Braasch WC. Surgical aspects of polycystic kidneys. Surg Gynecol Obstet 1934: 58: 649.  Back to cited text no. 1    
2.Zeir M, Gerberth S. Ritz E. ADPKD: Clinical problems. Nephron 1988:49:177.  Back to cited text no. 2    
3.Gregoire JR. Torres VE. Holley KE. Farrow GM. Renal epithelial hyperplastic and neoplastic proliferation in ADPKD. Am J Kidney Dis 1987: 9: 27.  Back to cited text no. 3    
4.Keith DS, Torsey VE. King BE. RCC in ADPKD. J Am Soc Nephrol 1994:4:1661.  Back to cited text no. 4    


    Figures

  [Figure - 1], [Figure - 2]



 

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    Introduction
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    Review
    References
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