title: Surgical treatment of localized renal cell carcinoma in von Hippel-Lindau's disease.
abstract: From 1981 to 1986, 10 patients with von Hippel-Lindau's disease underwent an operation for bilateral nonmetastatic renal cell carcinoma. Of the patients 9 underwent unilateral partial nephrectomy and contralateral radical nephrectomy, and 1 underwent bilateral nephrectomy with subsequent hemodialysis and cadaver renal transplantation. Pathologically, the tumor was stage I in 9 patients and stage III in 1. Currently, 8 patients are alive after partial nephrectomy (5 to 56 months) with good renal function (mean serum creatinine 2.0 mg. per dl.) and no evidence of malignancy; 1 of these patients underwent excision of a cerebral metastasis 2 years after partial nephrectomy. One patient is alive on dialysis after removal of the renal remnant for local tumor recurrence. The patient who underwent transplantation is free of tumor with a well functioning allograft. The distinctive features of renal cell carcinoma in von Hippel-Lindau's disease that influence the management of these patients are reviewed.
authors.affiliation: Department of Urology, Cleveland Clinic Foundation, Ohio 44106.
authors.forename: W F
authors.initials: WF
authors.lastname: Spencer
authors.forename: A C
authors.initials: AC
authors.lastname: Novick
authors.forename: J E
authors.initials: JE
authors.lastname: Montie
authors.forename: S B
authors.initials: SB
authors.lastname: Streem
authors.forename: H S
authors.initials: HS
authors.lastname: Levin
mesh_terms: D000328:Adult; D000368:Aged; D000798:Angiomatosis / Q000150:complications*; D002292:Carcinoma, Renal Cell / Q000150:complications / Q000473:pathology / Q000601:surgery*; D006801:Humans; D007680:Kidney Neoplasms / Q000150:complications / Q000473:pathology / Q000601:surgery*; D016030:Kidney Transplantation; D008875:Middle Aged; D009392:Nephrectomy; D006623:von Hippel-Lindau Disease / Q000150:complications*