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CASE REPORT
Year : 2004  |  Volume : 20  |  Issue : 2  |  Page : 58-59
 

Renal cell carcinoma in autosomal dominant polycystic kidney disease: A case report


Kolkata, India

Correspondence Address:
Subroto Ranjan Poddar
Dept. of Urology, New PG Qtrs - 102, SSKM Hospital, 242, AJC Bose Road
India
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Source of Support: None, Conflict of Interest: None


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Keywords: Renal tumor, Polcystic kidney, ADPKD, RCC, Haematuria


How to cite this article:
Poddar SR, Sinha SK, Chatterjee U, Kundu AK. Renal cell carcinoma in autosomal dominant polycystic kidney disease: A case report. Indian J Urol 2004;20:58-9

How to cite this URL:
Poddar SR, Sinha SK, Chatterjee U, Kundu AK. Renal cell carcinoma in autosomal dominant polycystic kidney disease: A case report. Indian J Urol [serial online] 2004 [cited 2019 Jun 26];20:58-9. Available from: http://www.indianjurol.com/text.asp?2004/20/2/58/37174



   Case Report Top


In June 2003, a 41-years old nondiabetic & normotensive male presented with intermittent gross total painless hematuria for last 6 months . During this period he had been treated for several febrile episodes. He had no family history for ADKPD. Examination revealed bilateral renomegaly and the right kidney was larger & harder than the left. Routine biochemical & haematological parameters were within normal limits. Renal ultra sonogram showed pancreatic, bilateral & multiple renal cysts and a large exophytic mass from right kidney with few areas of calcification & renal vein thrombus. CT scan confirmed the findings [Figure - 1],[Figure - 2] and [Figure - 3]. Diagnostic CT guided aspiration cytology was positive for malignant cells The patient was subjected to a right radical nephrectomy. Histopathology of nephrectomy specimen revealed Grade IV papillary & clear variety of renal cell carcinoma [Figure - 4] with renal vein thrombus in a polycystic kidney specimen. The postoperative period was uneventful. The patient is on follow up.


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ADPKD is the most common cystic disease seen in 1:5001000 patients [2] . The risk of renal carcinoma in these patient is controversial & reportedly less than 1% [3] . Moreover, ADKPD is not a risk factor for RCC, but in these patients RCC occurs at a younger age and there is increased incidence of high grade, bilateral, multricentric, sarcomatoid tumors. [4] There are no clinical features which could indicate the presence of RCC in ADPKD. CT scan characterizes any malignant mass in ADPKD cyst and a CT­guided FNABC of the cyst of interest would certainly give the reliable information. The majority of renal call carcinomas associated with ADPKD are clear cell type [5] . The case presented here showed mixed papillary and clear cell pattern. These tumors are hypovascular, less invasive and have a better prognosis when compared to the more vascular renal adenocarcinomas in normal kidney [5]. . Because of high incidence of metachronous tumor in ADKPD, the management of these type of unilateral tumor is slightly different from RCC in normal kidney. Some authors have followed up their patients with multiple imaging modalities after radical nephrectomy. These patients needs aggressive approach if any suspicious lesion seen in the remaining kidney [6] .

 
   References Top

1.Walters W, Braasch WE. Surgical aspects of polycystic kidney. Surg Gynecol Obstet. 1934; 58:649.  Back to cited text no. 1    
2.Zeir M, Gerberth S, Ritz E. ADKPD: Clinical problems. Nephron 1988; 49:177.  Back to cited text no. 2    
3.Gregoire JR, Torres VE, Holly KE, Farrow GM.Renal epithelial hyperplastic and neoplastic proliferation in ADKPD. Am J Kidney Dis 1987; 9:27.  Back to cited text no. 3    
4.Keith DS, Torsey VE, King BE. RCC in ADKPD. J Am Soc Nephrol 1994; 4:1661.  Back to cited text no. 4    
5.Mydio JH, Bard RH. Analysis of papillary renal adenocarcinoma. Urology 1987; 30: 529-534.  Back to cited text no. 5    
6.NG RCK, Suki WN. Renal cell carcinoma occurring in a polycystic kidney of a transplant recipient. J Urol 1980; 124:710-712.  Back to cited text no. 6    


    Figures

  [Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4]



 

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