Uscanga-Yépez J, Rodríguez-Covarrubias F, Morales J, Granados J, Gabilondo-Navarro F

Language: Spanish
References: 21
Page: 318-322
PDF size: 178.94 Kb.

ABSTRACT

Objective. To describe the clinical outcome of surgical treatment of renal-cell carcinoma in patients with von Hippel-Lindau disease. The clinical, demographic and histopathological characteristics were evaluated. Material and methods. Of 468 patients with renal-cell carcinoma treated surgically at our institution from 1982 to 2010, eight with von Hippel-Lindau disease were identified. Relevant variables included tumor stage, type of surgery, recurrence and mid-term renal function. Results. Seven out of eight patients 7 were treated with nephron-sparing surgery. The other underwent radical nephrectomy. During the follow-up of 89.3 ± 67.5 months, two patients had ipsilateral tumor ease.recurrence and one in the contralateral kidney. Mean survival was 72 months in seven out of eight patients that also preserved normal renal function; one patient with uncontrolled diabetes died five years after surgery from complications of chronic kidney disease. Conclusion. Nephron-sparing surgery offers acceptable functional and oncologic results for patients with renal-cell carcinoma and von Hippel-Lindau dis

REFERENCES

1. Matin SF, Ahrar K, Wood CG, Daniels M, Jonasch E. Patterns of intervention for renal lesions in von Hippel-Lindau disease. BJU Int 2008; 102: 940-5.

2. Maher ER, Neumann HP, Richard S. Von Hippel-Lindau disease: a clinical and scientific review. Eur J Hum Genet 2011; 19: 617-23.

3. Lonser RR, Glenn GM, Walther M, Chew EY, Libutti SK, Linehan WM, Oldfield EH. von Hippel-Lindau disease. Lancet 2003; 361: 2059-67.

4. Dollfus H, Massin P, Taupin P, et al. Ocular manifestations in von Hippel-Lindau disease: a clinical and molecular study. Invest Ophthalmol Vis Sci 2002; 43: 3067-74.

5. Wanebo JE, Lonser RR, Glenn GM, Oldfield EH. The natural history of hemangioblastomas of the central nervous system in patients with von Hippel-Lindau disease. J Neurosurg 2003; 98: 82-94.

6. Neumann HP, Bausch B, McWhinney SR, et al. Germ-line mutations in nonsyndromic pheochromocytoma. N Engl J Med 2002; 346: 1459-66.

7. Hammel PR, Vilgrain V, Terris B, et al. Pancreatic involvement in von Hippel-Lindau disease. The groupe francophone d’étude de la maladie de von Hippel-Lindau. Gastroenterology 2000; 119: 1087-95.

8. Maher ER, Kaelin WG Jr., Von Hippel-Lindau disease. Medicine (Baltimore) 1997; 76: 381-91.

9. Spencer WF, Novick AC, Montie JE, Streem SB, Levin HS. Surgical treatment of localized renal cell carcinoma in von Hippel- Lindau’s disease. J Urol 1988; 139: 507-9.

10. Steinbach F, Novick AC, Zincke H, et al. Treatment of renalcell carcinoma in von Hippel-Lindau disease: a multicenter study. J Urol 1995; 153: 1812-16.

11. Frydenberg M, Malek RS, Zincke H. Conservative renal surgery for renal cell carcinoma in von Hippel-Lindau’s disease. J Urol 1993; 149: 461-4.

12. Novick AC, Streem SB. Long-term follow up after nephron sparing surgery for renal cell carcinoma in von Hippel-Lindau disease. J Urol 1992; 147: 1488-90.

13. Roupret M, Hopirtean V, Mejean A, et al. Nephron sparing surgery for renal cell carcinoma and von Hippel-Lindau’s disease: a single center experience. J Urol 2003; 170: 1752-5.

14. Walther MM, Choyke PL, Glenn G, et al. Renal cancer in families with hereditary renal cancer: prospective analysis of a tumor size threshold for renal parenchymal sparing surgery. J Urol 1999; 161: 1475-9.

15. Herring JC, Enquist EG, Chernoff A, Linehan WM, Choyke PL, Walther MM. Parenchymal sparing surgery in patients with hereditary renal cell carcinoma: 10-year experience. J Urol 2001; 165: 777-81.

16. Jeschke K, Peschel R, Wakonig J, Schellander L, Bartsch G, Henning K. Laparoscopic nephron-sparing surgery for renal tumors. Urology 2001; 58: 688-92.

17. Guillonneau B, Bermúdez H, Gholami S, et al. Laparoscopic partial nephrectomy for renal tumor: single center experience comparing clamping and no clamping techniques of the renal vasculature. J Urol 2003; 169: 483-6.

18. Shingleton WB, Sewell PE Jr. Percutaneous renal cryoablation of renal tumors in patients with von Hippel-Lindau disease. J Urol 2002; 167: 1268-70.

19. Rendon RA, Kachura JR, Sweet JM, et al. The uncertainty of radio frequency treatment of renal cell carcinoma: findings at immediate and delayed nephrectomy. J Urol 2002; 167: 1587-92.

20. López-Garibay LA, Cendejas-Gómez JJ, Rodríguez-Covarrubias F, et al. Función renal a largo plazo en pacientes con cáncer de células renales tratados quirúrgicamente: comparación entre nefrectomía radical y parcial. Rev Invest Clin 2013; 65: 7-11.

21. Rasmussen A, Nava-Salazar S, Yescas P, et al. Von Hippel-Lindau disease germline mutations in Mexican patients with cerebellar hemangioblastoma. J Neurosurg 2006; 104: 389-94.