Indian Journal of Urology
CASE REPORT
Year
: 2002  |  Volume : 18  |  Issue : 2  |  Page : 150--151

Renal cell carcinoma in autosomal dominant polycystic kidney disease


M Raghavendran, Rakesh Kapoor, Anant Kumar, Waheed Zaman, Aneesh Srivastava, Anil Mandhani 
 Department of Urology, SGPGI, Lucknow, India

Correspondence Address:
Rakesh Kapoor
Department of Urology, SGPGIMS, Raebareli Road, Lucknow - 226 014
India




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;18:150-151


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 7 ];18:150-151
Available from: http://www.indianjurol.com/text.asp?2002/18/2/150/37619


Full Text

 Introduction



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



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



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 [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

1Walters W, Braasch WC. Surgical aspects of polycystic kidneys. Surg Gynecol Obstet 1934: 58: 649.
2Zeir M, Gerberth S. Ritz E. ADPKD: Clinical problems. Nephron 1988:49:177.
3Gregoire JR. Torres VE. Holley KE. Farrow GM. Renal epithelial hyperplastic and neoplastic proliferation in ADPKD. Am J Kidney Dis 1987: 9: 27.
4Keith DS, Torsey VE. King BE. RCC in ADPKD. J Am Soc Nephrol 1994:4:1661.