Ureteroscopic management of upper tract urothelial carcinoma (UTUC) in patients with Lynch Syndrome (hereditary nonpolyposis colorectal cancer syndrome)
A partir de données médicales portant sur 13 patients atteints d'un syndrome de Lynch et d'un carcinome urothélial des voies urinaires supérieures (âge moyen : 56,5 ans ; durée moyenne de suivi : 59 mois), cette étude évalue, du point de vue du taux de conservation rénale et du contrôle de la maladie, l'efficacité d'une résection urétéroscopique par laser
Objectives * To report our experience with ureteroscopic laser ablation of upper tract urothelial carcinoma (UTUC) in patients with Lynch Syndrome (LS), as defined by a documented germline mutation in the MSH-2 gene. * To increase awareness among urologists about UTUC in this unique patient population and refer to genetic counselling when appropriate. Patients and Methods * Demographic, clinical and pathological data on 13 consecutive patients with UTUC and documented MSH-2 mutation comprising 15 involved renal units were retrospectively collected. * Ureteroscopic evaluations involved biopsy and laser treatment with combination holmium/neodymium yttrium aluminum garnet (YAG) lasers. * Tumours were graded from 1 to 3 according to the 1973 World Health Organisation classification by a single pathologist evaluating cell block preparations. Results * The mean patient age at initial presentation was 56.5 years, with six of 13 patients having metachronous bilateral UT disease. * The mean follow-up was 59 months with a mean number of surveillances of 12. * Of 15 affected renal units, 10/15 (67%) of initial tumours involved the ureter with mean lesion size of 17.5 mm, while five of 15 (33%) involved the intrarenal collecting system with mean lesion size of 25 mm. * Ureteroscopy cleared 13/15 (87%) lesions and four of those 13 (31%) of those needed staged procedures. Renal preservation rate was 14/15 (93%) with one nephroureterectomy and one segmental ureterectomy performed. * One patient developed metastatic UTUC after 40 months surveillance. No patient presented with bladder tumours but seven of the 13 (54%) developed them within 10 months of the initial ureteroscopy. Conclusions * Patients with LS who develop UTUC present at younger ages and appear to be more likely to have bilateral UT disease over their lifetimes vs sporadic UTUC patients. * Ureteroscopic laser ablation offers a good renal preservation rate with reasonable cancer control in patients willing to undergo endoscopic surveillance. * Development of new bladder tumours is common.
http://onlinelibrary.wiley.com/doi/10.1111/bju.12008/abstract