Surgical procedures for Von Hi ppel-Li ndau syndro me acco mpanied with renal cell carci no ma (9 cases report)
Li Shic hao
Huang Qingbo
Gong Huijie
Gao Yu
Zhang Yu
Zhang Xu
Ma Xin
Abstract：Objective :To expl ore the surgical procedures for Von Hi ppel-Li ndau (VHL )syndro me acco mpanied with renal cell carci no ma (RCC ).Methods :Fro m March 2009 to August 201 3 ,9 cases of VHL syndro me acco mpa-nied with RCC were subj ected to treat ments i n PLA General Hospital .Fa mil y history ,cli nical manifestati ons ,char-acteristics of RCC and surgical treat ments were retrospecti vel y studied .Results :Most of the patients were 20-40 year ol d ,and 4 out of 9 cases had si gnificant fa mil y history of central nervous syste m (CNS )he mangi oblasto ma .Ho wev-er ,the rest had CNS he mangi oblasto ma without si gnificant fa mil y history .Besi des RCC ,this ki nd of syndro me i n-cl uded pancreatic tu mor or pancreatic cyst ,adrenal pheochro mocyto ma ,etc .All of RCC were bilateral or multi-focus clear cell RCC .The grade and stage of these RCC were l o w.Nephron spari ng surgery was co mmon surgical treat-ment for these groups ,i ncl udi ng laparoscopic and open approaches .Ni ne patients with RCC under went 1 0 opera-ti ons ,with a mean operati on ti me of 1 42 .5 mi n ,mean bl ood l oss of 1 63 .3 mL and mean hospital stay of 7 .2 days . Conclusions :VHL syndro me acco mpanied with RCC is an i nherited disease with multi ple lesi ons .Surgical operati on is the first choice for treat ment of renal tu mors ,and laparoscopic nephron spari ng operati on is safe ,reasonable and effecti ve treat ment for this ki nd of syndro me .
Keywords：Von Hi ppel-Li ndau syndro meki dney neoplas msi nherited diseasessurgical treat ment
Publication Date：2015-01-01
Online Publishing Date：2025-08-15(First online date of this platform, not the publication date of the document)
Pages：4( 71-74 )