Interdisciplinary Management of Renal Cell Carcinoma Associated to Von Hippel-Lindau Disease

Daruich M, García Marchiñena P, Jurado A, Gueglio G
Rev. Arg. de Urol. · Vol. 81 (3) 2016

Abstract

OBJECTIVES:

We aimed to describe the clinical characteristics of patients with Von Hippel-Lindau disease, evaluated by the “Von Hippel-Lindau disease interdisciplinary management group” of a Latin American hospital, noting the management and behavior of renal cell carcinoma associated with this syndrome.

MATERIALS AND METHODS:

A retrospective observational descriptive study was conducted. We included all patients with clinical diagnosis of this disease evaluated during the period from August 2014 to August 2015.

RESULTS:

Eleven patients were included. Mean age of first manifestation was 30 (17-56) years. The initial clinical manifestations were hypertension (54.5% [n=4]), neurological symptoms (18.1% [n=2]), eye symptoms (18.1% [n=2]) and others (27.2% [n=3]). A family history was detected in 81.8% (n=9) of cases. The 54.5% (n=6) of the patients developed renal cell carcinoma with an average of 2.5 (1-5) surgeries per patient. The 81.8% (n=9) of these procedures were open procedures. The mean resected tumors per procedure was 3 (1-8). One patient began hemodialysis and another patient developed metastasis. Mean follow- up was 7.5 (1-26) years.

CONCLUSIONS:

The interdisciplinary management of renal cell carcinoma associated with Von Hippel-Lindau disease could prevent and differ the sequelae associated, not only to the loss of kidney function, but also in other organs involved in the disease, improving the quality of life of these patients.

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