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Q1: What are the differential diagnoses?
The most important differential diagnosis to consider in a young patient with multiple colorectal polyps is that of familial adenomatous polyposis. Other conditions that might be considered are hyperplastic polyposis coli, Peutz-Jeghers syndrome, multiple juvenile polyposis, Gardener's syndrome, Cronkhite-Canada syndrome, Turcot's syndrome, Cowden's syndrome, and pseudopolyposis secondary to ulcerative colitis.
Q2: What is the diagnosis for these patients?
Hyperplastic polyposis coli.
Q3: How would you manage patients with this diagnosis?
Regular colonoscopy with polypectomy may be sufficient. Prophylactic colectomy, as would be advocated in familial adenomatous polyposis, is probably not warranted in this condition unless a secondary carcinoma is detected.
Hyperplastic polyps of the colon are common benign lesions. They frequently arise at the crest of a mucosal fold where they present as small, sessile polyps usually measuring less than 5 mm in diameter with a smooth convex surface.1 Although traditionally regarded as non-neoplastic, recent evidence has shown hyperplastic polyps to have molecular features of neoplasia.2
Histologically they have a very characteristic appearance of elongated crypts with prominent epithelial infoldings giving a pathognomonic serrated pattern. The majority of hyperplastic polyps occur in the rectosigmoid where they may be single or often multiple; however their numbers rarely exceed 10.1 The occurrence of multiple or large hyperplastic polyps in the large bowel is termed hyperplastic polyposis coli.1 3 4
Hyperplastic polyposis coli is a rare condition characterised by the presence of large multiple hyperplastic polyps, some of which may show dysplasia, an occurrence that is felt to contribute significantly to the observation that approximately 40% of sporadic cases are associated with adenocarcinoma.1 Hyperplastic polyposis coli has recently been more clearly defined by Jørgensenet al as “at least 20 polyps distributed evenly or segmentally in the colorectum or at least two polyps larger than 10 mm in diameter”.1 Place et al have advanced a different definition of what they call hyperplastic adenomatous polyposis syndrome.5 They define this as at least 50 polyps, including adenomatous polyps, greater than 5 mm in diameter and associated with adenocarcinoma of the colon. Unfortunately this definition excludes the majority of cases reported in the literature as hyperplastic polyposis syndrome. One family has also been reported with eight affected individuals in three generations, six of whom had adenocarcinoma.4 Only one of these eight cases fulfil the criteria advanced by Jørgensenet al.1
The dysplasia seen in these polyps (see fig 1) is that of a mixed hyperplastic-adenomatous polyp showing areas of unequivocal benign hyperplastic morphology and areas of unequivocal dysplastic adenomatous morphology within the same polyp. This pattern is different from serrated adenoma where the entire polyp shows dysplasia within a polyp showing a serrated growth pattern.2 3 Hyperplastic polyposis coli should be distinguished from serrated adenomatous polyposis as suggested by Torlakovic et al.3
There is an important clinical implication for hyperplastic polyposis coli. Clinically this syndrome can resemble familial adenomatous polyposis and clearly needs to be distinguished, as the treatment for the uncomplicated syndrome would be radically different for each of these two disorders. There is a significant risk of cancer for hyperplastic polyposis coli patients with approximately 40% of the cases reported in the literature, that is 23 of the 52 cases, associated with adenocarcinoma. This is an entirely different proposition to that of familial adenomatous polyposis with its attendant 100% risk of cancer for the 12/100 000 who are born with this mutation. Adequate follow up is important with regular colonoscopy being the investigation of choice, but segmental colectomy may be considered necessary in instances where there is a segmental increase in large polyps. However prophylactic total colectomy, as advocated in cases of familial adenomatous polyposis, may not be warranted.1
Hyperplastic polyposis coli.
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