Abstract
Background: Bilateral renal cell carcinoma (RCC) exists in hereditary forms (von Hippel-Lindau disease, hereditary papillary renal cell carcinoma, and hereditary clear cell renal carcinoma) associated with various chromosomal abnormalities, and non-hereditary, apparently sporadic forms. The focus of this study is the clinical description of the latter entity.
Methods: Synchronous and asynchronous bilateral RCC were identified from a prospective database of 698 consecutive patients undergoing operation for RCC between July 1989 and December 1997 at Memorial Sloan-Kettering Cancer Center. Non-familial RCC was defined as that occurring in those patients without a family or hereditary history of RCC. Patients' records were evaluated for presentation, surgical approach used, and pathology. Actuarial survival from the date of initial operative treatment until the date of last follow-up or death was determined using the Kaplan-Meier method. Comparisons between groups were made using the Mann-Whitney test.
Results: Thirty-three of 698 (4.7%) patients operated for RCC had bilateral disease. Four of the 33 (12.1%) patients had either VHL or documented hereditary RCC, and 29 of 33 (87.9%) had non-familial RCC. Of the 29 patients, histology was conventional (clear cell) in 17 patients, papillary in 5, oncocytoma in 3, and unclassified in 3. One patient had a conventional (clear cell) histology in the first nephrectomy specimen and chromophobe renal cell carcinoma in the second. Partial nephrectomy was used in 100% of patients. Median follow-up time was 52 months. Actuarial 5-year overall survival was 84.5%, and actuarial disease-specific survival was 93.3% at 5 years for the non-familial RCC patients.
Conclusions: Non-familial bilateral RCC patients represent a distinct subpopulation of renal cancer patients with a good overall prognosis. Partial nephrectomy is an integral part of the surgical management. Although most bilateral tumors present synchronously, asynchronous lesions may occur many years after original nephrectomy, thus committing the patient to long-term follow-up.
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References
McDonald MW. Current therapy for renal cell carcinoma.J Urol 1982;127:211–7.
Linehan WM, Lerman MI, Zbar B. Identification of the von Hippel-Lindau (VHL) gene. Its role in renal cancer.JAMA 1995;273:564–70.
Gnarra JR, Glenn GM, Latif F, Anglard P, Lerman MI, Zbar B, Linehan WM. Molecular genetic studies of sporadic and familial renal cell carcinoma.Urol Clin North Am 1993;20:207–16.
Lubensky IA, Gnarra JR, Bertheau P, Walther MM, Linehan WM, Zhuang Z. Allelic deletions of the VHL gene detected in multiple microscopic clear cell renal lesions in von Hippel-Lindau disease patients.Am J Pathol 1996;149:2089–94.
Kaplan E, Meier P. Nonparametric estimation from incomplete observations.J Am Stat Assoc 1958;53:457–81.
Zhuang Z, Gnarra JR, Dudley CF, Zbar B, Linehan WM, Lubensky IA. Detection of von Hippel-Lindau disease gene mutations in paraffin-embedded sporadic renal cell carcinoma specimens.Mod Pathol 1996;9:838–42.
Gnarra JR, Tory K, Weng Y, et al. Mutations of the VHL tumour suppressor gene in renal carcinoma.Nature Genetics 1994;7:85–90.
Anglard P, Tory K, Brauch H, et al. Molecular analysis of genetic changes in the origin and development of renal cell carcinoma.Cancer Res 1991;51:1071–7.
Licht MR, Novick AC, Goormastic M. Nephron sparing surgery in incidental versus suspected renal cell carcinoma.J Urol 1994;152:39–42.
Campbell SC, Novick AC, Streem SB, Klein E, Licht M. Complications of nephron sparing surgery for renal tumors.J Urol 1994;151:1177–80.
Provet J, Tessler A, Brown J, Golimbu M, Bosniak M, Morales P. Partial nephrectomy for renal cell carcinoma: indications, results and implications.J Urol 1991;145:472–6.
Lerner SE, Hawkins CA, Blute ML, Grabner A, Wollan PC, Eickholt JT, Zincke H. Disease outcome in patients with low stage renal cell carcinoma treated with nephron sparing or radical surgery.J Urol 1996;155:1868–73.
Morgan WR, Zincke H. Progression and survival after renal-conserving surgery for renal cell carcinoma: experience in 104 patients and extended followup.J Urol 1990;144:852–7.
Bennington JL, Beckwith JB. Renal adenocarcinoma. In: Firminger HI, ed.Tumors of the Kidney, Renal Pelvis, and Ureter. Washington, DC: Armed Forces Institute of Pathology, 1975:81–200.
Franklin JR, Figlin R, Belldegrun A. Renal cell carcinoma: basic biology and clinical behavior.Semin Urol Oncol 1996;14:208–15.
Zbar B, Tory K, Merino M, et al. Hereditary papillary renal cell carcinoma.J Urol 1994;151:561–6.
Zbar B, Glenn G, Lubensky IA, et al. Hereditary papillary renal cell carcinoma: clinical studies in 10 families.J Urol 1995;153:907–12.
Perez-Ordonez B, Hamed G, Campbell S, Erlandson RA, Russo P, Gaudin PB, Reuter VE. Renal oncocytoma: a clinicopathologic study of 70 cases.Am J Surg Pathol 1997;21:871–83.
van den Berg E, Dijkhuizen T, Storkel S, et al. Chromosomal changes in renal oncocytomas.Cancer Genet Cytogenet 1995;79:164–8.
Bryant DA, Scheinfeld AG, Russo P, Gaudin PB, Reuter VE. Conventional renal cell carcinoma: a clinicopathologic study of 183 cases from 1991 to 1994.Modern Pathol 1998;11:77A.
Reuter VE, Scheinfeld AG, Hamed G, Campbell S, Gaudin P, Russo P. Papillary renal cell carcinoma: a clinicopathologic study of 83 tumors.Am J Surg Pathol (in press).
Hamed G, Perez-Ordonez B, Russo P, Gaudin PB, Reuter VE. Chromophobe renal cell carcinoma: A clinicopathologic study of 51 cases.Modern Pathol 1996;9:74A.
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Grimaldi, G., Reuter, V. & Russo, P. Bilateral non-familial renal cell carcinoma. Annals of Surgical Oncology 5, 548–552 (1998). https://doi.org/10.1007/BF02303649
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DOI: https://doi.org/10.1007/BF02303649