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

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.

Coexistence of Granular Cell Tumor and Invasive Ductal Breast Cancer in Contralateral Breasts: A Case Report

“…Primary tumors that coexist at the time of diagnosis are defined “synchronous” and this condition is quite common for malignant lesions that occur in bilaterally symmetrical organs, such as breasts [ 3 ], kidneys [ 6 ], ovaries [ 7 ], and testes [ 8 ].…”

Review of renal tumors associated with Birt-Hogg-Dubé syndrome with focus on clinical and pathobiological aspects

“…The same as renal tumors of other familial syndrome are managed [14], mere close observation should be undertaken until the largest tumor size reaches 3 cm. When the largest tumor exceeds 3 to 4 cm in the largest diameter, nephron-sparing surgery should be considered [12,14,25,[64][65][66]. If partial nephrectomy is not technically suitable, onetime radical nephrectomy should be performed.…”