Elsevier

Urology

Volume 21, Issue 2, February 1983, Pages 146-150
Urology

Scientific article
Diagnosis and management of asymptomatic renal cell carcinomas in von Hippel-Lindau syndrome

https://doi.org/10.1016/0090-4295(83)90010-9Get rights and content

Abstract

Renal cell carcinomas occurred in 6 of 17 subjects with the von Hippel-Lindau syndrome (HLS). In 4 patients, the tumors were discovered by abdominal computed tomography (CT) during a prospective study. Abdominal CT is recommended as a safe and accurate method for screening patients with HLS for renal cell carcinoma. Renal cell carcinoma in HLS tends to be multifocal and bilateral. Because of this, it is recommended that the tumors be treated by surgical enucleation rather than by more extensive surgical procedures which might ultimately lead to the need for dialysis.

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    There are no specific guidelines regarding the type or timing of surgery in patients who have VHL disease. Many management strategies have been proposed, such as observation, nephrectomy, and NSS, depending on the size, number, and location of the tumors and the rate of recurrence.17,18,79,88,96,97 Currently, the treatment options available for hereditary forms of renal cancer are observation with no planned intervention but periodic imaging every 6 to 12 months to monitor tumor growth; NSS to remove discrete renal tumors while sparing normal renal tissue if tumors increase to 3 cm or larger; and unilateral or bilateral nephrectomy to eliminate the risk of developing metastatic cancer.9,11,94

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