[Surgical treatment of multiple polyps of the large intestine (review of the literature)].
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Incidence and mortality of the carcinoma of the large intestine increase also in the GDR. Carcinomas mostly develop in adenomas of the large intestine in the course of several years. Nowadays the adenoma-carcinoma-sequence is regarded as ascertained. The environmental influences are of decisive importance for the genesis of the colon carcinoma. Apart from this also genetic factors play a part. Villous adenomas more frequently show malignant structures than tubular adenomas. In large adenomas with a diameter of more than 20 mm more frequently invasive carcinomas (20-40%) are found than in small adenomas with a diameter of less than 10 mm (1%). From this is to be derived the demand of the removal of all adenomas of the large intestine with a diameter larger than 5 mm. The method of choice of the treatment is the polypectomy and the technique with the diathermy loop.
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We report on a girl with hamartomatous intestinal polyps and a large mass that involved most of the posterior wall of the bladder, which on microscopic examination had the characteristics of a hamartoma. Hamartomas are among the rarest of bladder tumors. Our case is only the third reported under this designation, although 3 other cases may fall into this category. Of the 6 patients 4 have been children. Although most polypoid bladder tumors in children are rhabdomyosarcomas of the botryoid type, our case illustrates that rarely other lesions are similar grossly. Recognition of these lesions has clinical implications with regard to therapy and prognosis.
The distribution of polyps of the large intestine was investigated. Comparisons were made between results of autopsy studies and a clinical study consisting of 114 consecutively resected polyps. The shift from distal to proximal predominance of adenomas with increasing age from the 50 to the 80 year age group in autopsy studies, was in accordance with the high frequency of adenomas in the distal part of the large intestine in the prospective clinical study, mean 60 years of age. The present clinical study showed a high frequency of adenomas among men, whereas a tendency towards larger polyps and more polyps larger than 10 mm was seen for women. The present examination confirms the reported discrepancy between a high prevalence of adenomas compared to a much lower prevalence of cancer. Thus, the present results emphasize the need for further studies to gain information on risk factors in polyps or individuals predisposed for malignancy of the large intestine.
The management of patients with endoscopically removed malignant intestinal polyps is controversial. The risk of residual disease should be assessed against the risk of a surgical operation. The authors report 35 cases of malignant polyps (5.5% of 641 colonoscopically removed adenomas). Sixteen patients had carcinoma in situ and received no further treatment and 19 had invasive carcinoma (sessile in 6, pedunculated in 13). Of these 19, 7 did not undergo surgery--because of old age in 2, minimal invasion in 3, a low rectal location in 1 and refusal in 1. Twelve patients (3 with sessile, 9 with pedunculated polyps) underwent a surgical resection, and residual disease was present in 3 (25%), 1 with positive nodes. Reported criteria of increased risk of residual disease--cancer in lymphatics or veins, incomplete excision, tumour at resection margin, sessile and villous tumours--were present in nine. All three patients with residual disease had microscopically involved margins of resection. The authors believe that the increased risk of recurrence justifies the risk associated with subsequent surgical resection unless the patient is otherwise a poor operative risk.
The clinical and pathologic features of 64 inflammatory fibroid polyps of the large and small bowel in 63 patients are reported. Six of the lesions in the small bowel were studied by electron microscopy. Follow-up information was obtained from 39 patients. The lesion was always benign, occurred at all ages, and had a world-wide distribution. The lesions were polypoid, sessile, originated in the submucosa, infiltrated the muscularis propria, and most closely resembled granulation tissue. The principal mesenchymal cell by electron microscopy was the fibroblast. No associated medical conditions were identified and the cause remains unknown.
Polyps of distal portions of the large intestine were revealed by rectoromanoscopic examination in 268 patients. In 109 of them solitary and group polyps were found by a colanoscopic examination in proximal portions of ileum, 14 having malignant polyps. In addition, 11 patients had malignant tumors. Polyps in the proximal portions are often found in patients older than 60.
Mutations in the human adenomatous polyposis coli (APC) gene are responsible for not only familial adenomatous polyposis but also many sporadic cancers of the digestive tract. Using homologous recombination in embryonic stem cells, we recently constructed Apc gene knockout mice that contained a truncation mutation at codon 716 (Apc(delta716)). The heterozygous mice developed numerous intestinal polyps. All microadenomas dissected from nascent polyps had already lost the wild-type allele, indicating the loss of heterozygosity (M. Oshima et al., Proc. Natl. Acad. Sci. USA, 92: 4482-4486, 1995). We also demonstrated that cyclooxygenase 2 is induced in the polyps at an early stage and plays a key role in polyp development (M. Oshima et al., Cell 87: 803-809, 1996). We have analyzed the process of polyp development in these mice both at morphological and molecular levels. A small intestinal microadenoma is initiated as an outpocketing pouch in a single crypt and develops into the inner (lacteal) side of a neighboring villus forming a double-layer nascent polyp. The microadenoma then enlarges and gets folded inside the villus. When it fills the intravillous space, it expands downward and extends into adjoining villi, rather than rupturing into the intestinal lumen. During this course of development, the basement membrane remains intact, and the labeling index of the microadenoma cells is similar to that of the normal crypt epithelium. As in the crypt cells, neither transforming growth factor beta1 nor its receptor type II is expressed in the microadenoma cells. No hot spot mutations in the K-ras gene are found in the microadenoma tissue during these early stages of polyp development. Essentially, the same results have been obtained for the colonic polyps as well. These results suggest that early adenomas in the Apc(delta716) polyps are very similar to the normal proliferating cells of the crypt except for the lack of directed migration along the crypt-villus axis.
A 30-year-old woman developed proptosis secondary to a left ethmoidal compact osteoma. At age 29 years, a mandibular eburnated (ivory) osteoma was excised. At age 25 years, multiple adenomatous polyps of the colon were resected. Her father, age 61 years, had multiple intestinal polyps and bilateral mandibular osteoma. A 24-year-old sister had an osteoma of the forehead. Gardner's syndrome is an autosomal dominantly inherited disorder characterized by intestinal polyposis, various skin and soft tissue tumors, and osteomas of the bony skeleton. Orbital osteomas occur rarely.
BACKGROUND: It has been recently documented that multiple bilateral pigmented lesions at the level of the retinal pigment epithelium may be an indicator of patients with familial adenomatous polyposis who are prone to develop intestinal cancer, particularly if there is a positive family history of these intestinal disorders. Although atypical, such lesions have been called congenital hypertrophy of the retinal pigment epithelium (CHRPE). This study was undertaken to determine whether the typical lesions of CHRPE, seen frequently by ophthalmologists, also were indicators of familial adenomatous polyposis. METHODS: Review of charts and follow-up studies were performed on all patients diagnosed and coded as having solitary CHRPE or its multifocal variant (congenital grouped pigmentation; bear tracks). Patients and their physicians were contacted by telephone to complete a detailed questionnaire designed to detect signs or symptoms of familial adenomatous polyposis or Gardner syndrome among these patients with CHRPE and their relatives. RESULTS: Of the 132 patients with previously diagnosed CHRPE, there were none with familial adenomatous polyposis, Gardner syndrome, or intestinal cancer, and only one patient had a history of intestinal polyps. Among more than 2000 of their blood relatives, only 20 had intestinal polyposis or colonic cancer (1%). This is much lower than would be expected from a survey of patients with the typical fundus lesions seen with familial adenomatous polyposis. CONCLUSIONS: It appears that solitary CHRPE and congenital grouped pigmentation differ clinically from the multiple pigmented lesions seen with familial adenomatous polyposis and that patients with these conditions, as well as their relatives, are not at a greater risk of developing intestinal cancer.
Aggregation chimeras were formed between C57BL/6 mice heterozygous for the Apc(min) (Min) mutation and wild-type SWR mice, that differ in their Pla2g2a status, a modifier of Apc(min), and also in their resistance to intestinal polyp formation. Variation in the dolichos biflorus agglutinin-staining patterns of the intestines of these mouse strains was used to determine the chimeric composition of the intestine in individual mice and to examine the clonal composition of adenomas. Macroscopic adenoma numbers in chimeric mice were compared with the expected adenoma numbers based on the percentage of C57BL/6J-Apc(min/+) epithelium in individual mice. These results unexpectedly show that there was no apparent inhibitory effect of the SWR-derived (Pla2g2a wild-type) tissue on adenoma formation in the C57BL/6J-Apc(min/+) epithelium. This suggests that the main genetic modifiers of the Min phenotype act at a cellular or crypt-restricted level with no discernable systemic effect. All adenomas were seen to contain C57BL/6J-Apc(min/+)-derived epithelium, confirming that the germ-line mutation of the mApc gene is necessary to initiate tumorigenesis in this model system, and that the mApc gene acts in a cell autonomous fashion.
Peutz-Jeghers syndrome is a rare hereditary disease, although in about 20 % of patients there is no known family history. Its clinical hallmarks are facial mucocutaneous pigmentation and diffuse gastrointestinal polyposis of hamartomatous origin. The major difficulty in the management of this disease lies in the complications of surgery for small bowel polyposis and the high incidence of tumors presented by these patients as adults. We present the case of a 10-year-old girl with treatment-resistant anemia and no other clinical symptoms. Further investigation revealed pigmentation in the oral mucosa and polyposis in the stomach and small intestine. Hamartomatous lesions were confirmed by histological study of intestinal biopsy. No familial antecedents of pigmentation or intestinal polyps were found. During evolution the patient required subtotal resection of the small intestine due to invagination.
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Partly obstructing, proliferative mucosal masses in the small intestine of two dogs were shown histologically to be hamartomatous polyps. They were characterized by an extension of smooth muscle from the muscularis mucosae into the lamina propria of the lesion. This is the first report of such lesions in domestic animals. In one of the dogs the lesion had become malignant.