Abstract
4152 Background: PAC in BC survivors is rare (Andersson, Acta Oncologica, 2008, 47:755-764) and may occur as part of an inherited cancer predisposition syndrome. Up to 10% of PAC patients (pts) have an inherited form of the disease with germline mutations (m) in BRCA1 and BRCA2 representing a portion of this group (Lynch, Pancreatology, 2001. 1:466-471). PALB2 has recently been identified as a susceptibility gene for both BC and PAC (Jones, Science, 2009, Apr 10; 324:217). Clinical outcomes in BC survivors who develop PAC have not been previously described. Methods: 30 pts with a personal history of BC and PAC were identified from the MSKCC Pancreatic Cancer Family Registry; 13 pts (43%) were Jewish. Two pts had undergone testing for BRCA m, both negative. Six other pts with BRCA2 m and PAC (1 male) and one pt with BRCA1 m and PAC, were also identified. Results: Of 30 pts with BC and PAC, median age at diagnosis of PAC and BC was 66.5 years (yrs) and 61 yrs, respectively. Median interval between diagnosis of BC and PAC was 7.5 yrs. Eight (27%) pts had a first-degree relative (FDR) with BC; 6 (20%) pts had a FDR with colon cancer; and 2(7%) had a FDR with PAC (Table). Conclusions: OS in BC survivors with PAC appears greater than predicted for all stages of disease. In pts with a personal history of BC and PC, genetic testing for germline m in BRCA1 and BRCA2 as well as consideration of PALB2 testing is appropriate. Further genetic characterization of this group may help to influence treatment choices and guide prognosis. The use of platinum therapy, RT, and more recently PARP inhibitors to target the BRCA-associated defective DNA repair mechanism, are deserving of further evaluation in pts with BRCA associated PAC. BC and PAC (n=30) BRCA and PAC (n=7) Median age at diagnosis 66.5 yrs 60 yrs OS resected 53.3 months (mo) (n=10) 1 pt deceased 19.2 mo 2 pts alive at 27.2 and 2.3 mo post op OS locally advanced (LA) 24.2 mo (n=7) 2 pts alive at 9.8 and 8.7 mo OS metastatic (M1) 11.4 mo (n=13) 18.3 mo (n=2) # of pts receiving platinum chemo for LA or M1 PAC n=10 (of 27) n= 4 (of 6)* # of pts receiving radiotherapy (RT) for LA PAC n=5 (of 7)** n= 2 * 1 pt achieved CR. ** 1 pt had subsequent R0 resection. No significant financial relationships to disclose.
Published Version
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have