Scientific paperRectal cancer in hereditary nonpolyposis colorectal cancer
Section snippets
Patients and methods
A retrospective medical records review was undertaken of HNPCC patients with documented rectal cancer between December 1948 and December 1999. The study population was selected from patients with documented pathology in the Roswell Park Cancer Institute (RPCI) HNPCC registry. This registry includes those patients who have documented germline mutations in mismatch repair genes, meet the Amsterdam criteria, or have a strong clinical history suggestive of HNPCC. Clinically, 5 patients had
Results
Eighty-nine patients in the registry had a total of 139 documented colorectal adenocarcinoma. Twenty-five of the 89 (28%) patients were diagnosed with rectal adenocarcinoma. The median age at diagnosis of rectal cancer was 50 years (range 24 to 79). There were 6 women (24%) and 19 men (76%). Twenty-one of the 25 (84%) patients with rectal adenocarcinoma met the Amsterdam criteria for HNPCC [12]. The remaining 4 patients (16%) had germline mutations in hMLH1 or hMSH2. Eight of 25 (30%) patients
Comments
Right-sided colorectal cancer predominance is one of the characteristics of HNPCC patients. Nevertheless, there is a small, yet significant incidence of left-sided carcinomas. The incidence of right-sided lesions has been reported as high as 70% [7]. However, as patients treated with less than abdominal colectomy are followed up, the difference decreases. At our institution we reported a 60% incidence of right-sided neoplasms at presentation, but on subsequent follow-up, the incidence was 52%
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