Hyperplastic Polyposis Syndrome

5/26/2004

Question: Is there a syndrome of hyperplastic polyps, and what is its relationship to colorectal cancer?

<4>

Unique Identifier:10930367

Authors: Rashid A. Houlihan PS. Booker S. Petersen GM. Giardiello FM. Hamilton SR.

Institution: Department of Pathology, The Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.

Title: Phenotypic and molecular characteristics of hyperplastic polyposis.

Source: Gastroenterology. 119(2):323-32, 2000 Aug.

Abstract: BACKGROUND & AIMS: Patients with hyperplastic polyposis are reported to have multiple and/or large hyperplastic polyps (HPs) and an increased risk of colorectal cancer, but the phenotype and genetic alterations in hyperplastic polyposis have not been studied in detail. METHODS: We evaluated clinical-pathological and molecular characteristics of 129 HPs, 6 serrated adenomas, and 3 admixed hyperplastic-adenomatous polyps from 13 patients with hyperplastic polyposis (more than 20 HPs), 5 patients with a large HP (>/=1 cm in diameter), and 5 patients with multiple HPs (5-10 HPs). RESULTS: HPs in the right colon in contrast to the left colorectum had more frequent topographic dysregulation of p21(Waf-1/Cip1) expression (94% vs. 76%, P = 0.03) and of proliferation (92% vs. 53%, P = 0. 0001), but less frequent allelic loss of chromosome 1p (4% vs. 17%, P = 0.03). K-ras mutation was present in 8% of HPs, p53 gene product overexpression in none, and microsatellite instability in 3% without relationship to microsatellite instability in synchronous cancer. Patients with a large HP differed from those with multiple HPs in having a high frequency of right-sided HP (63% vs. 22%, P = 0.01) and of right-sided colon cancer (100% vs. 8%, P = 0.003). Hyperplastic polyposis was associated with a family history of colorectal cancer (P = 0.01) and with loss of chromosome 1p in HP (21% vs. 0%, P = 0.001). CONCLUSIONS: A hyperplastic polyp/dysplasia-to-adenocarcinoma sequence can be manifested in 3 distinct phenotypes consisting of patients with hyperplastic polyposis and chromosome 1p allelic loss in some HPs, in contrast to patients who have large, right-sided HPs or small numbers of HPs that lack 1p loss. CAS Registry/EC Number 0 (Cip1 protein). 0 (Cyclins). 0 (Protein p53).

<5>

Unique Identifier:10235578

Authors: Place RJ. Simmang CL.

Institution: Department of Surgery, University of Texas Southwestern Medical Center, Dallas 75235-9156, USA.

Title: Hyperplastic-adenomatous polyposis syndrome. [Review] [27 refs]

Source: Journal of the American College of Surgeons. 188(5):503-7, 1999 May.

Abstract: BACKGROUND: Although the syndrome of familial adenomatous polyposis is well known, sporadic patients with multiple polyposis are rare. There are no known syndromes associated with hyperplastic polyposis. In our search of the English surgical literature, we find no reference to a hyperplastic-adenomatous polyposis syndrome. STUDY DESIGN: Over a 3-year period, we identified six patients ages 41 to 75 (mean age 61) with 50 to 100 hyperplastic polyps associated with adenomas. RESULTS: Most of the hyperplastic polyps were found in the left colon and the largest ranged in size from 6 mm to 18 mm. The larger polyps were clinically indistinguishable from adenomas. Three of our six patients had invasive cancer of the proximal colon. All tumors were confined to the bowel wall. There was a family history of colon cancer in only one patient and no family history of polyposis. CONCLUSION: These patients differ from previously described patients with polyposis syndromes; hyperplastic-adenomatous polyposis syndrome (HAPS) occurs in an older population with no family history of polyposis, has fewer polyps, most of which are hyperplastic, and is strongly associated with adenocarcinoma of the colon. In this series, we describe a previously unreported hyperplastic-adenomatous polyposis syndrome. [References: 27]

<7>

Unique Identifier:8691339

Authors: Jeevaratnam P. Cottier DS. Browett PJ. Van De Water NS. Pokos V. Jass JR.

Institution: Department of Pathology, University of Auckland School of Medicine, New Zealand.

Title: Familial giant hyperplastic polyposis predisposing to colorectal cancer: a new hereditary bowel cancer syndrome.

Source: Journal of Pathology. 179(1):20-5, 1996 May.

Abstract: A mother and five of her ten offspring developed colonic cancers, the mother and one of the offspring being younger than 50 years of age at diagnosis. Despite fulfilling the Amsterdam criteria for hereditary non-polyposis colorectal cancer (HNPCC), several features pointed towards the possibility that this represented a different syndrome of familial cancer. Most notable was the presence of large, multiple hyperplastic polyps and mixed polyps in four of the subjects whose pathology was available for review. In addition, three of the four subjects had cancers that were negative for DNA replication errors (RER-). The subject with an RER+ cancer had a second RER+ cancer and three adenomas, one in contiguity with the second cancer. This subject also had multiple, large hyperplastic polyps, thereby combining hyperplastic polyposis and a proneness to multiple RER+ tumours. One of the hyperplastic polyps was also RER+. Two of five young asymptomatic descendants have been found to harbour multiple colorectal polyps. It is suggested that giant hyperplastic polyposis is a new familial syndrome predisposing to colorectal cancer. CAS Registry/EC Number 0 (DNA, Neoplasm).