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
Department of Urology, SGPGIMS, Raebareli Road, Lucknow - 226 014
|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 2020 Jan 20 ];18:150-151
Available from: http://www.indianjurol.com/text.asp?2002/18/2/150/37619
Walters and Braasch first reported association of ADPKD and RCC in 1934, and 30 such cases have been described in the literature.  Hereby we present one such case seen.
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.
ADPKD is the most common cystic disease seen in 1: 500-1000 patients.  Risk of renal carcinoma in these patients is controversial and reportedly  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.  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.
|1||Walters W, Braasch WC. Surgical aspects of polycystic kidneys. Surg Gynecol Obstet 1934: 58: 649.|
|2||Zeir M, Gerberth S. Ritz E. ADPKD: Clinical problems. Nephron 1988:49:177.|
|3||Gregoire JR. Torres VE. Holley KE. Farrow GM. Renal epithelial hyperplastic and neoplastic proliferation in ADPKD. Am J Kidney Dis 1987: 9: 27.|
|4||Keith DS, Torsey VE. King BE. RCC in ADPKD. J Am Soc Nephrol 1994:4:1661.|