Multifocal renal oncocytoma in a patient with Von Hippel-Lindau mutation.
Von Hippel-Lindau disease (VHL) is a rare genetic disease with a lifetime risk of clear cell renal cell carcinoma in approximately 70% of cases. We present a case of a 63-year-old man with bilateral, multifocal renal masses. Genetic testing results were consistent with a VHL deletion. The patient had no other disease manifestations consistent with VHL. The patient underwent staged bilateral nephron-sparing procedures. Pathology of all renal masses revealed oncocytoma. To our knowledge, we describe the first reported case of multiple renal oncocytomas in a male patient with a germline VHL mutation.
Fiske
Joshua
J
Department of Urology, New York University School of Medicine/Bellevue Hospital, New York, New York, USA.
Patel
Rupa
R
Kau
Eric
E
Pappas
John G
JG
Garcia
Roberto A
RA
Taneja
Samir S
SS
D018249:Adenoma, Oxyphilic / Q000235:genetics*; D006801:Humans; D007680:Kidney Neoplasms / Q000235:genetics*; D008297:Male; D008875:Middle Aged; D009154:Mutation; D006623:von Hippel-Lindau Disease / Q000235:genetics*