Staged, Open, No-Ischemia Nephron-Sparing Surgery for Bilateral-Multiple Kidney Tumors in a Patient with Birt-Hogg-Dubé Syndrome

Metin Vural,Ahmet Musaoglu,Fatin Cezayirli,Kamil Peker,Terman Gümüş,Ayşe Deniz Akkaya,Tarık Esen,Ahmet Tefekli

doi:10.1155/2012/639629

Abstract

Hereditary kidney cancer patients with bilateral multiple kidney tumors represent challenges in the era of rapidly growing minimal invasive treatment techniques. Birt-Hogg-Dubé Syndrome (BHDS) is an autosomal dominant genodermatosis characterized by a triad of benign skin tumors (fibrofolliculomas, trichodiscomas, acrochordons) together with an increased risk of developing malignant renal tumors and pulmonary disease such as pneumothoraces and multiple lung cysts. The morbidity and mortality of the affected patients is determined by the presence of the kidney tumors, which tend to be multifocal and bilateral, as observed in other hereditary kidney cancer syndromes like von Hippel-Lindau disease, familial leiomyomatosis, and hereditary papillary renal cell carcinoma. Herein, a patient with BHDS, presenting with synchronous bilateral multiple kidney tumors, is reported. The report describes the management of kidney tumors with two-stage open nephron-sparing surgery in which the nonvascular clamping technique was utilized.

Highlights

Birt-Hogg-Dube Syndrome (BHDS) is an autosomal dominantly inherited genodermatosis caused as a result of the mutations in the FLCN gene encoding the protein “folliculin,” a new protein which hypothetically functions as a tumor suppressor gene [1,2,3,4]
Kidney tumors of the patient were managed with two-staged open nephron-sparing surgery (NSS), and we describe our noischemia surgical technique with the kidney under perfusion
There is a debate going on regarding the technique of applying renal ischemia during NSS to avoid renal damage in the treated kidney [10]

Summary

Case Report

Open, No-Ischemia Nephron-Sparing Surgery for Bilateral-Multiple Kidney Tumors in a Patient with Birt-Hogg-Dube Syndrome. Hereditary kidney cancer patients with bilateral multiple kidney tumors represent challenges in the era of rapidly growing minimal invasive treatment techniques. The morbidity and mortality of the affected patients is determined by the presence of the kidney tumors, which tend to be multifocal and bilateral, as observed in other hereditary kidney cancer syndromes like von Hippel-Lindau disease, familial leiomyomatosis, and hereditary papillary renal cell carcinoma. A patient with BHDS, presenting with synchronous bilateral multiple kidney tumors, is reported. The report describes the management of kidney tumors with two-stage open nephron-sparing surgery in which the nonvascular clamping technique was utilized

Introduction

Discussion