Joseph X. Jenkins

Colorectal adenomas in the lynch syndromes. Results of a colonoscopy screening program

Forty-four asymptomatic putative Lynch syndrome patients participated in a colonoscopy screening program. There were 18 men and 26 women; mean age was 44 yr. Thirty percent of Lynch syndrome patients had at least one adenoma; 20% had multiple adenomas. In 18% of the patients, adenomas were discovered proximal to the splenic flexure. In a reference group of 88 age- and sex-matched patients, 11% had adenomas, 4% had multiple adenomas, and 1% had right-sided adenomas. Twenty-one Lynch syndrome patients had follow-up colonoscopies. Of 7 patients with adenomas on initial examinations, 6 had adenomas at follow-up. Of 14 patients with negative initial examination results, 3 had adenomas at follow-up. The prevalence of adenomas in the Lynch syndromes is greater than in an unselected reference group. The adenomas are more proximally located, corresponding to the site of cancer distribution in the Lynch syndromes. A high rate of synchronous and metachronous lesions is found. Our findings support the hypothesis that adenomatous changes are the premalignant lesion in the Lynch syndromes. We also found evidence of heterogeneity among Lynch syndrome families in adenoma incidence.

Hereditary flat adenoma syndrome: A variant of familial adenomatous polyposis?

We describe the clinical and pathologic features in four extended kindreds that are consistent with the hereditary flat adenoma syndrome (HFAS). This colon cancer susceptibility disorder is believed to be inherited as an autosomal dominant. The principal phenotypic marker is multiple colonic adenomas (usually less than 100), with a tendency for proximal location. The majority of these adenomas are flat or slightly raised and plaquelike, as opposed to polypoid. Colon cancers have typically developed in middle age and show no unusual histologic features. There are a variety of extracolonic manifestations, including adenomas and carcinomas of the small bowel and fundic gland polyps. The HFAS is contrasted with hereditary nonpolyposis colorectal cancer and familial adenomatous polyposis (FAP) and shown to be distinct from both in the numbers and distribution of colonic adenomas and the typical age of cancer diagnosis. The clinical implications of these findings are discussed. Given its linkage to the FAP locus on 5q and the phenotypic parallels between HFAS and FAP, we conclude that HFAS is a variant of FAP.

Epidemiologic characteristics of the flat adenoma of Muto. A prospective study.

The flat adenoma is an endoscopically visible lesion that histologically consists of adenomatous change near the luminal surface of colonic tubules. We have described three families with hereditary colon cancer with later age of onset than familial adenomatous polyposis (FAP) and with multiple proximal colonic flat adenomas. These families have been linked to the FAP locus on chromosome 5. Our aim was to determine whether the flat adenoma is pathognomonic of this hereditary flat adenoma syndrome (HFAS) or merely an atypical or early tubular adenoma with occurrence in patients other than those from colon cancer-prone families. Methods: We prospectively examined a population referred for colonoscopy within a one-year period. During colonoscopy, flat adenomas were specifically sought and all lesions were removed endoscopically and evaluated histologically. Members of known hereditary colon cancer families were excluded. Results: One hundred forty-eight patients underwent colonoscopy (64 men and 84 women). Median age was 61 years. Fifty-seven patients had 157 polyps. One hundred thirty-six polyps were reviewed histologically. Thirty-five (23.6 percent) of the referred patients had adenomas, of whom twelve patients had only flat adenomas while six had both flat and other adenomas (18=12 percent of 148). The associations between flat adenoma occurrence and various predictors (sex, race, prior colonic neoplasms, family history of cancer, synchronous adenomas) were similar to those seen with other adenomas. Flat adenomas were found in nearly equal proportions of patients under or over age 61 years (11 percent and 13 percent, respectively). Other adenomas were significantly more common in the older group (6 percentvs.25 percent;P<0.002 by Fishers exact test). Conclusion: In a referral practice, the flat adenoma has the same prevalence and associated risk factors as other adenomas, except for younger age of onset. Our data suggest that the flat adenoma represents an early stage of adenoma development that is manifested in a subset of patients from the general population and that, as an isolated event, does not provide a marker for a hereditary colon cancer-prone syndrome.

Upper gastrointestinal manifestations in families with hereditary flat adenoma syndrome.

Background. The hereditary flat adenoma syndrome (HFAS) is characterized by an autosomal dominantly inherited predisposition to multiple colonic adenomas (usually less than 100) with proximal predominance and flat as opposed to polypoid growth. Patients with the syndrome experience colorectal cancers in excess, and the lesions are distributed randomly in the colon. The polyps occur at a later age (median, 55 years) compared with age at onset of polyps in patients with familial adenomatous polyposis (FAP) and patients with the Lynch syndromes. FAP and HFAS are linked to the same locus on chromosome 5q21‐q22.

Open laparoscopy for laparoscopic cholecystectomy.

This report deals with the first 350 laparoscopic cholecystectomies performed at Creighton University, beginning in October 1989, by one of the authors (R.J.F., Jr.). An open technique was used for initial access to the abdominal cavity in 343 patients (98%). Patients included 274 females and 76 males, with an average age of 45.5 years. Weight ranged from 72 to 316 pounds (32–142.2 kg). The laparoscopic procedure was successfully completed in 338 (96.6%). Operative cholangiography was performed in 113 (32.3%), and 52 (14.7%) had acute cholecystitis as determined by the final pathological report. Median operating time was 80 min. There was no operative mortality. Bile duct injury was not seen. Postoperative complications occurred in 21 patients (6%), only two of which were directly related to the open technique (cellulitis of the umbilical wound requiring antibiotics). A third patient (0.3% of 343 successful laparoscopies) required reoperation on postoperative day 4 for a small bowel perforation; the exact cause could not be determined, but conceivably it was related to the umbilical minilaparotomy. We conclude that the open technique is safe and effective method for initial peritoneal access prior to laparoscopic cholecystectomy.

Colonoscopy Findings in a Series of Patients with Hereditary Colorectal Cancer

During a colonoscopy screening program for patients at high risk for hereditary nonpolyposis colorectal cancer (HNPCC), we observed one family which was atypical in that members showed right-sided flat adenomas. The total colonic adenoma counts exceeded those found in HNPCC, but were fewer than that characteristically observed in familial adenomatous polyposis (FAP). There was a high rate of synchronous and metachronous adenomas. These observations highlight the heterogeneity in HNPCC and stress the need for careful delineation of all facets of the colonic phenotype in the interest of etiologic classification of hereditary colorectal cancer syndromes.