Abstract
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.
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%)
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.
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