Ces Urol 2007, 11(2):93-97 | DOI: 10.48095/cccu2007009

Renal carcinoma in patients with von Hippel Lindau disease

T. Ürge¹, M. Hora¹, P. Toufarová¹, O. Hes², I. Šubrt³, Z. Chudáček⁴, B. Kreuzberg⁵

¹ Urologická klinika LF UK a FN, Plzeň

² Šiklův patalogicko-anatomický ústav LF UK a FN, Plzeň

³ Ústav lékařské genetiky LF UK a FN, Plzeň

⁴ Radiodiagnostické oddělení LF UK a FN, Plzeň

⁵ Radiologická klinika LF UK a FN, Plzeň

Objective: Von Hippel Lindau disease is autosomal dominant disorder (VHL) with prevalence 1/39000-1/50000. Its characteristic sign is tumours infliction of different organs. Kidneys infliction occurs at 59-63% patients, from which 24-45% forms are clear cell renal carcinoma (CRCC). Authors present three cases with clinical aspects.

Methods: 1 240 patients were surgically treated in dept. of urology since January 1991 to November 2006, three patients (0.24%) were affected with VHL disease.

Results: Case 1: Thirty seven-years old man was examined for instability, giddiness, hearing noise in the year 1992. He underwent resection of cerebellum in 1994 on the left side and extirpation of hemangioblastoma (HGB) and inguinal orchiectomyon the left side for duct papillary adenoma of spermatic cord.
Within 1999 he underwent transperitoneal nephrectomy on the left side for CRCC and 6 months later we performed resection of right kidney for tumor duplicity. In 2002 was performed metastasis extirpation of CRCC from left retroperitoneal space and in the year 2004 we performed re-operation of right kidney for recurrence of disorder. Finally in October 2006 laparoscopic assisted radiofrequency ablation of tumour in front lip of solitary kidney was performed.
Case 2: At sixty four, years old man we performed in the year 2005 transperitoneal nephrectomy and contralateral resection of CRCC. Recurrence of disorder occurred 12 months later. Nephrectomy was offered to him. He rejected it.
Case 3: Thirty eight years old woman underwent cerebelotomy for cystic HGB in the year 1993. In years 1997 and 2004 she was reoperated for recurrence of this disorder. In 2005 bilaterally salpingectomy and extirpation of left ovarium for cystadenoma and bilateral transperitoneal nephrecromy for CRCC was performed. All cases were examinated for genetic abnormality. Patients follow-up is 17-168 (median 92 months).

Keywords: von Hippel Lindau, hemangioblastoma, renal carcinoma

Published: March 1, 2007