Spectrum of Renal Pathology in Patients with Congenital Renal Anomalies – A Case Series from a Tertiary Cancer Center
Jasreman Dhillon, Sambit K Mohanty, Tim Kim, Wade J Sexton, Philippe E Spiess. Moffitt Cancer Center, Tampa, FL
Background: Various congenital renal anomalies (CRA) may be detected in adults. These are classified into anomalies in number, size, position, form (fusion) and structure. Some of the CRAs detected in adults include horseshoe kidney (HSK), crossed fused renal ectopia (CFRE) and malrotation. CRA are rare and renal lesions associated with CRA even rarer. We present a series of cases from our institution documenting the pathology of renal lesions with CRAs from 2005 upto 2012.
Design: Since 2005, 11 patients were referred with renal masses in context of CRA. Patients underwent partial or radical nephrectomy. Presenting symptoms, demographics, pathology and modalities of management and outcomes from 9 cases of HSK and 2 cases of CFRE were reviewed.
Results: Patients included 10 males and 1 female. The mean age at diagnosis was 57 years (37 to 76 years). Presenting symptoms were gross (n=3) and microscopic hematuria (n=2), flank pain (n=4) and asymptomatic (n=2). Patients were treated with partial (n=9) and radical (n=4) nephrectomies. Pathology ranged from benign (1 simple cortical cyst and 1 chronic pyelonephritis with uretero-pelvic junction obstruction and secondary hydronephrosis) to malignant (11 cases of renal cell carcinomas). 2 patients with HSKs presented with bilateral renal masses; one received preoperative neoadjuvent Sunitinib and later underwent right radical nephrectomy and left partial nephrectomy for bilateral clear cell renal cell carcinoma (RCC) and the second underwent right and left partial nephrectomies for a simple cortical cyst and a papillary, type 1 RCC. RCCs ranged in size from 2.5 cm to 13 cm. There were 10 cases of clear cell RCC and 1 case of papillary type 1 RCC. Fuhrman nuclear grades were 3 (n=5), 2 (n=5) and 1 (n=1). 9 cases were stage pT1 and 2 were pT2. Two patients with CFRE developed clear cell RCC. All cases had negative surgical margins. Follow-up, available in all the patients, ranged from 1 month to 49 months. None of the patients developed any recurrences or metastases.
Conclusions: Renal pathology in HSK and CFRE of the kidney is presented. Most of the cases occur in men. Our study confirms the findings that RCCs are the most common tumors presenting in this clinical setting, with clear cell RCC being the most common histologic subtype. We also observed that in adult patients, the most common congenital anomaly associated with RCC is HSK. All the tumors behave in an indolent fashion with prognosis related to tumor stage. Partial nephrectomy is a safe and effective procedure in appropriately selected patients.
Category: Genitourinary (including renal tumors)
Monday, March 4, 2013 1:00 PM
Poster Session II # 165, Monday Afternoon