AUA2016: Characterization of upper tract urothelial carcinoma in patients with clinical suspicion of Lynch syndrome

AUA2016 Annual Meeting

 Saturday, May 7, 2016 8:00 AM-10:00 AM
SDCC: Room 30DE

Bladder Cancer: Upper Tract Transitional Cell Carcinoma I
Funding: None

PD13-07: Characterization of upper tract urothelial carcinoma in patients with clinical suspicion of Lynch syndrome
  Abstract: PD13-07
 
Introduction and Objectives
Lynch syndrome (LS) is the only known hereditary cancer syndrome associated with upper tract urothelial carcinoma (UTUC). Patients with LS have a reported 14-fold increase in relative risk of developing UTUC compared to the general population. The prevalence and clinical implications of potential hereditary UTUC are not well understood due to the rarity of this clinical entity and the lack of awareness among urologists. The goal of this study was to evaluate the prevalence and outcome of UTUC in patients with LS-associated cancers.

Methods
We analyzed UTUC cases (primary site codes C65.9 and 66.9 with transition cell carcinoma histology) in the Surveillance, Epidemiology, and End Results (SEER-17) database. LS-associated cancers were identified in patients with UTUC. Based on the revised Bethesda Guidelines for Lynch Syndrome, LS-associated cancers include colorectal, endometrial/uterine, gastric, ovarian, small bowel, glioblastoma, sebaceous adenocarcinoma, biliary tract, and pancreatic cancers. UTUC-specific survival was compared between patients with UTUC alone and those with LS-associated cancers.

Results
Between 1973 and 2011, 26,819 cases of UTUC were reported in SEER. A total of 1731 (6.4%) of cases of UTUC also had at least one LS-associated cancer. Colorectal, uterine, and ovarian cancers were the most prevalent LS-associated cancers, accounting for 4.3%, 2.2%, and 0.7% of UTUC cases respectively. Compared to UTUC-only cases, those with LS-associated cancers have higher UTUC-specific survival when controlled for disease stage and age of diagnosis (p<0.0001).

Conclusions
The risk of developing UTUC in LS may be underappreciated. Patients with potential hereditary UTUC may have a different clinical course and survival outcomes compared to those with UTUC without LS-associated cancers. Further study and genetic evaluation are warranted.

Date & Time: May 7, 2016 8:00 AM-10:00 AM
Session Title: Bladder Cancer: Upper Tract Transitional Cell Carcinoma I
Sources of Funding: None