Multiple renal cell carcinoma in solitary kidney

MULTIPLE RENAL CELL CARCINOMA IN SOLITARY KIDNEY*

GARY B . BOKINSKY, M .D . MITCHELL GOLDMAN, M .D . From the Department of Urology and Army-Navy Transplant Service, Naval Medical Research Center, Bethesda, Maryland

ABSTRACT - An unusual case of asynchronous bilateral renal cell carcinoma presenting as multiple tumors in a solitary kidney is presented . A literature review suggests that the prognosis is improved for those patients with a longer interval between tumors and for those who undergo surgical therapy .

Renal cell carcinoma accounts for approximately 1 .2 per cent of all malignancies and 89 per cent of all renal tumors .' The treatment of the unilateral neoplasm is well established . Conversely, bilateral renal tumors or tumors occurring in a solitary kidney present more of a challenge, and the therapeutic alternatives are worth reviewing. We herein present a case of asynchronous bilateral renal cell carcinoma with multiple tumors in the solitary kidney after a fifteen-year interval . Case Report A fifty-four-year-old white woman underwent a left radical nephrectomy followed by irradiation to the operative field in 1962 . Pathologic examination revealed clear cell carcinoma, Stage 2 . Postoperatively she was considered tumor free until October, 1977, when routine intravenous pyelogram disclosed an irregular border to the right lower pole . Subsequent arteriogram showed two distinct hypervascular masses, one of which was located centrally near the renal hilum (Fig . 1) . Metastatic survey was otherwise negative, and the pathologic slides from 1962 were reviewed to eliminate angiomyolipoma from the differential diagnosis . *The views expressed herein are those of the authors and do not necessarily reflect views of the United States Navy or Department of Defense .

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FIGURE 1 . Arteriogram of solitary kidney showing two distinct hypervascular masses, one located centrally near renal hilum .

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FIGURE 2 . Bench surgery in solitary kidney : (A) multiple tumors, and (B) after wedging out tumor including adequate margins .

On October 31, 1977, the patient underwent a right radical nephrectomy through an extraperitoneal thoracoabdominal (tenth) rib approach . Mannitol (12 .5 Gm .) was infused prior to clamping the vessels, and the kidney was removed along with a cuff of vena cava . The kidney was immediately flush perfused with chilled Collins solution, surrounded by saline ice slush, and placed on the operative bench . The ureter was left in situ . While on the bench, three distinct tumors were wedged out with adequate margins (Fig . 2) . All calyceal and vascular repairs were done under direct vision, and the kidney was transplanted to the ipsilateral iliac fossa in the routine manner . Total cold ischemic time was approximately forty minutes . Urinary output was immediate and sustained, and renal function was unaltered . Postoperatively, her serum creatinine was 1 .0 mg./100 ml ., and creatinine clearance was 80 cc ./min . compared with preoperative values of 1 .1 mg ./100 ml . and 86 cc ./ min ., respectively . She was discharged on the eighth postoperative day, and has now been . followed up for one year . Her follow-up evaluation, including IVP and CAT scan, reveals no evidence of disease and stable renal function . Comment Relatively few cases of renal cell carcinoma in solitary kidneys have been reported, and the incidence of bilateral asynchronous renal tumor

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is even less frequent . In separate reviews Wickham' discussed 51 cases of tumors in solitary kidneys, and Grabstald and Aviles 3 another 45. Of these, 53 and 42 per cent, respectively, occurred after the contralateral kidneys were removed for malignant disease . Generally men were affected more commonly than women (28 :16),4 and most tumors were located in one of the renal poles.' The question of whether asynchronous tumors represent metastases or a new primary remains speculative . Wickham2 theorized that bilateral synchronous and asynchronous tumors represent a spectrum of the same process supporting probable metastatic disease . He believes that the interval between presentations of the bilateral lesions represents the degree of tumor aggressiveness, with synchronous tumors being the most aggressive . In his collective review, he showed that patients with bilateral synchronous tumors, which may be seen with the HippelLindau syndrome,' had a mortality of 72 per cent within six months . Surgery remains the preferred treatment since both radiation and chemotherapy have been shown to be ineffective . Aggressive treatment with radical nephrectomy and chronic hemodialysis or homotransplantation as primary procedures seems to offer suboptimal results . The inherent disadvantages of the altered quality of life, economic burden, and dependence on drugs and artificial life supports are obvious . Additionally, the estimated three-year survival of persons on chronic UROLOGY

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dialysis in the age groups where most of the bilateral tumors occur is between 44 and 47 per cent.' Cadaveric renal transplantation implicates the inherent risk of tumor enhancement in the immunosuppressed patient and has only a 50 .6 per cent five-year patient survival in the same age group. 10 Stroup et al ." reported a 60 per cent survival after radical nephrectomy and transplantation with a mean follow-up of less than two years . Further survival data are not yet available . Partial nephrectomy, either by in situ or extracorporeal means, appears to be the procedure of choice . In respective literature reviews, Novick et al . 5 reported 67 per cent survival after operative therapy as opposed to only 17 per cent survival in patients treated nonoperatively . Wickham' similarly showed 75 per cent operative survival and only 25 per cent survival after no treatment. Grabstald and Aviles 3 reported 23 of 30 patients to survive from one to ten years after partial nephrectomy, and Malek and Greene 4 had 6 of 7 patients survive a mean of 3 .8 years . Partial nephrectomy is performed usually in situ since this is technically the easiest method since most tumors are localized to the renal poles . In situ excision may be enhanced by local circulatory arrest and hypothermia, allowing up to three hours of ischemic time ." Care must be taken to provide adequate tumor-free margins, excellent hemostasis, and closure of the collecting systems . Renal function may be protected by adequate preoperative hydration and intraoperative mannitol infusions prior to clamping the renal vessels . When tumors are centrally located and in situ dissection is considered potentially hazardous, extracorporeal bench surgery may be used, as in our case where the perihilar lesion would have been difficult to excise . An additional advantage would be the ability to do angiography on the bench and the theoretical advantage of less risk of tumor spillage to the renal bed . It is not necessary to divide the ureter in most cases which negates the need for ureteroneocystostomy . Simple flush perfusion and hypothermia are adequate for short-term preservation ." Therefore in most cases, we do not

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believe that there is any particular advantage to pulsatile perfusion as has been suggested by others . 14 Summary The incidence of bilateral renal cell carcinoma, either synchronous or asynchronous, is between 1 .8 and 3 .8 per cent." The therapeutic approach should be tailored according to the natural history of this disease and its extent . Those patients that are treated surgically appear to have a better prognosis than those untreated or treated by other methods . Partial nephrectomy, either by in situ or ex vivo means, remains the preferred treatment . Chronic hemodialysis and transplantation have inherent disadvantages and should he avoided if possible . 5855 Bremo Road Richmond, Virginia 23226 (DR. BOKINSKY) References 1 . Bennington JL : Cancer of the kidney ; etiology, epidemiology, and pathology, Cancer 32 : 1017 (1973). 2 . Wickham JEA : Conservative renal surgery for adenocarcinoma, the place for bench surgery, Br . J . Urol . 47 : 25 (1975) . 3 . Grabstald H, and Aviles E : Renal cell cancer in the solitary or sole-functioning kidney, Cancer 22 : 973 (1968) . 4 . Malek RS, et al: Malignant tumors of solitary kidneys, Mayo Clin . Proc . 47 : 180 (1972). 5 . Novick AC, Stewart BH, Straffon RA, and Banowsky LH ; Partial nephrectomy in treatment of renal adenocarcinoma, J . Uml. 118 : 932 (1977). 6 . Males RS, and Greene LF : Urologic aspects of HippelLindau syndrome, ibid . 106: 800 (1971) . 7 . Vaeth JM : Cancer of the kidney : radiation therapy and its indications in non-Wilms tumors, Cancer 32 : 1053 (1973) . 8 . Talley RW : Chemotherapy in adenocarcinoma of the kidney, ibid . 32:1062 (1973) . 9 . Samuels S, Charra B, Olheiser K, and Blagg CR : Twelve years' experience of treatment of chronic renal failure, Trans. Am . Sm . Artific. Organs 20 : 62 (1974). 10 . The Twelfth Report of the Human Transplant Registry, JAMA 233 : 787 (1975) . 11 . Stroup BE, Shearer JK, Traurig AR, and Lytton B : Bilateral adenocarcinoma of the kidney treated by nephrectomy : a case report and review of the literature, J . Urol . 111 : 272 (1974). 12. Smith MJV, and Boyce WH : Anatmphic nephrotomy and plastic calyrhaphy, ibid . 99 : 521 (1968) . 13 . Bokinsky GB, Tester JH, and Concodora JA : Simplified renal preservation, Urology 10: 207 (1977) . 14. Cities RE, and McCullough D: Bench surgery in a solitary kidney, J . Urol . 112 : 12 (1975) . 15 . Viets DH, Vaughan ED, and Howards 55 : Experience gained from the management of 9 cases of bilateral renal cell carcinoma, ibid. 118: 937 (1977).

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