Rectal cancer in patients with hereditary nonpolyposis colorectal cancer: Surgical management and survival outcomes
© Nancy You et al; licensee BioMed Central Ltd. 2011
Published: 10 March 2011
Hereditary nonpolyposis colorectal cancer (HNPCC) is hallmarked by microsatellite instability. The prognosis of HNPCC-related colon cancer is well characterized, but little is known about rectal cancers. The aim of this study was to report the long-term outcomes of HNPCC-related rectal cancer where current-era multimodality therapy was utilized.
Patients referred to our institution for either primary or recurrent rectal cancer between 1992-2010 were identified based on following inclusion criteria: 1) pathogenic germline mutation in DNA mismatch repair genes (MMR; n=19); 2) germline variants of uncertain significance but tumor studies suggestive of MMR (n=6); 3) suggestive tumor studies but negative germline testing (n=5); and 4) suggestive tumor studies but no germline testing (n=4). Patients were reviewed for clinical characteristics and treatments, and followed to death or last contact.
After neoadjuvant (n=17)
No neoadjuvant (n=17)
Rectal cancer may present as the index cancer for HNPCC over a wide age range. Despite advanced stages at presentation, excellent long-term prognosis can be expected with aggressive multimodality therapy. Vigilant surveillance for recurrent or metachronous CRC should be carried out over a prolonged time period to allow for repeat surgical salvage and preserved long-term survival.
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