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A case of Gardners syndrome is described. This illness, inherited by autosomal dominance clinically shows multiple soft tumours, osteotomatosis, occasionally also odeontomata and polyposis intestinalis. The almost invariably occuring malignant degeneration of single intestinal polyps has led to the inclusion of Gardner's syndrome into the group of precancerous conditions.
The development and progression of colorectal cancer were studied by examining the mode of proliferation of cancer and adenoma in 806 cases (857 lesions) of colorectal cancers and 12 cases of familial polyposis coli. Colorectal cancer was classified into two groups: those accompanied by intramucosal growth or with polypoid growth and those without polypoid growth. Early cancer in the group without polypoid growth was considered to originate from de novo carcinoma of about 5 mm, showing vascular invasion when reaching the size of about 10 mm and then exhibiting massive submucosal invasion. Their cut-surface closely simulated cancer of ulcerative type without polypoid growth which occupied the greater portion of advanced cancer of the large bowel. In other words, ulcerative type cancer without polypoid growth is considered to arise from de novo carcinoma and this type occupies 70-90% of all colorectal cancers. Since the lesions originating from de novo carcinoma develops from an extremely small intramucosal carcinoma and then leads to a massive submucosal growth, immediate partial resection is recommended.
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Bowel obstruction is a rare but serious complication of pregnancy for both the mother and the developing fetus. This report describes the case of 17-year-old girl with ileal pouch-anal anastomosis (IPAA). She presented at 36 weeks' gestation with a complete small-bowel obstruction. Because conservative management was unsuccessful, labour was induced to relieve the obstruction or simplify surgery. Soon after spontaneous vaginal delivery she began to pass copious amounts of flatus and stool. The bowel obstruction resolved within hours. This report illustrates how IPAA alters the anatomy of the gastrointestinal tract, placing the ileal pouch at risk from compressive obstruction by the gravid uterus. Induction of labour in a near-term fetus is a reasonable initial method of management in such women.
Unlike colorectal cancer, risk markers for adenocarcinoma of the small intestine (ASI) have not been identified. Because the demographic and pathological features of both of these diseases are similar, immunohistochemistry was performed using monoclonal antibodies for three colonic premalignant markers, Adnab-9 (recognizes a colonic adenoma epitope), CaCo3/61, and FBB2/29 (small intestine proteoglycans expressed ectopically in colonic neoplasms), in normal and neoplastic small intestinal epithelium, and the results were compared with normal controls. Adnab-9 was also examined in 20 familial adenomatous polyposis (FAP) patients, a population known to be at an increased risk for ASI. Immunohistochemistry in normal and neoplastic tissue (adenoma, adenocarcinoma) from 18 patients with primary adenocarcinoma of the small intestine was compared with normal small intestine from 10 nonneoplastic controls. Four of 10 (40%) cases of normal small intestinal epithelium from controls were mildly positive in less than 10% of crypts, versus strong staining (>50% of crypts) in 16 of 18 (89%) patients with adenocarcinoma, and in 17 of 20 (85%) patients with FAP (P<.05). Adnab-9 predominantly stained Paneth cells as well as rare crypt and basal villous goblet cells. Adenomatous epithelium from the adenocarcinoma cases and adenomas from the FAP patients showed staining of Adnab-9 in 63% and 78% of cases, respectively. Only 17% of adenocarcinomas were positive for Adnab-9. In contrast, neither CaCo3/61 nor FBB2/29 showed any significant differences in the degree of staining in normal small intestinal epithelium in patients with adenocarcinoma compared with controls. Enhanced Adnab-9 staining in normal small intestinal epithelium from patients who harbor adenocarcinoma, and in FAP patients, supports its role as a risk marker of small intestinal neoplasia.
A 38-year-old man with a history of colonic and small bowel polyposis and glioblastoma was investigated for dyspepsia. Upper GI endoscopy identified an abnormal area in the duodenum, confirmed by histology as high grade non-Hodgkin's B cell MALT lymphoma. Although cases of Turcot's syndrome (TS) (colonic polyposis and primary brain tumour occurring in the same patient) have been previously described, association with haematological malignancy is rare. This is the first report of intestinal lymphoma occurring in an adult with TS.
Patients with familial adenomatous polyposis have an excess risk for adenomas and cancers of the upper and lower gastrointestinal tract. In the upper intestine these lesions occur mainly around the ampulla of Vater and they parallel mucosal exposure to bile. In view of this finding and of evidence that bile acids play a role in colorectal carcinogenesis, biliary bile acid profiles were determined in 29 patients with familial adenomatous polyposis (12 before colectomy, 17 after colectomy) and in 28 patients without familial adenomatous polyposis (all with colons in situ). Patients with familial adenomatous polyposis had a higher total biliary bile acid concentration than the others. The bile of patients with polyposis had a greater proportion of chenodeoxycholic acid and a lower proportion of deoxycholic acid than did the bile of patients without polyposis. The ratio of chenodeoxycholic acid and its metabolite lithocholic acid to cholic acid and its metabolite deoxycholic acid, which is related to subsequent bile acid profiles in the colon, was higher in patients with polyposis. Because bile acids influence cellular proliferation, these findings may be of importance with respect to intestinal adenoma and cancer growth.
BACKGROUND & AIMS: Mutations in the APC gene result in an increased propensity to develop intestinal neoplasia; however, a complete understanding of the mechanisms resulting in tumor formation has remained elusive. Min mice possess a mutation in the APC gene and display a neoplastic phenotype similar to that observed in familial adenomatous polyposis coli in humans. Cyclooxygenase (COX) inhibitors decrease tumor multiplicity in the Min mouse intestine. The present study was designed to determine if there was an increase in COX-2 in adenomas harvested from Min mouse intestine. METHODS: COX-2 messenger RNA levels were determined by Northern blots and reverse-transcription polymerase chain reactions of B6Min x 129 mouse-derived tumors. Protein levels and localization were determined by Western blots and immunohistochemical staining. RESULTS: The Northern blots revealed an approximately threefold increase in the level of COX-2 messenger RNA in Min mouse adenoma compared with normal mucosa. COX-2 protein levels in adenomatous tissues were also approximately threefold higher compared with normal mucosa from the same mouse. Immunohistochemical staining with a monospecific COX-2 antibody confirmed that increases in COX-2 immunoreactivity were restricted to dysplastic and neoplastic foci within intestinal mucosa. CONCLUSIONS: These data show that COX-2 levels may be increased at an early stage in colorectal neoplasia during polyp formation and before invasion.
We investigated whether the activity of ornithine decarboxylase might serve as a diagnostic test for detecting the presence of the genotype for familial polyposis. This rate-limiting enzyme in the polyamine biosynthetic pathway is essential for intestinal mucosal proliferation. In colonic mucosa from 16 normal controls, ornithine decarboxylase activity was less than 2.5 nmol per milligram per hour. In contrast, it was higher than 2.5 nmol per milligram per hour in the normal-appearing areas of colonic mucosa from 11 of 13 patients with familial polyposis and in all polyps biopsied from these same subjects (P less than 0.05 for specimens from both sites, as compared with controls). Mucosa from dysplastic polyps showed higher mean ornithine decarboxylase activity than mucosa from polyps that were not dysplastic (P less than 0.05). In colonic mucosa from clinically unaffected, first-degree relatives of patients with familial polyposis, there was a bimodal distribution of ornithine decarboxylase activity, with one peak at the mean for normal controls and the other near the mean for normal-appearing mucosa from affected patients. Our study suggests that ornithine decarboxylase activity in colonic mucosa may reflect the abnormal proliferative state in familial polyposis and identify clinically normal family members who carry the genotype.
PURPOSE: A case of a patient with familial adenomatous polyposis (FAP) is reported, in whom adenomas developed in an ileal pelvic pouch six years after it was made. This case is reported to serve as a warning that restorative proctocolectomy, a relatively recent addition to surgical options for FAP, does not remove the risk of metachronous intestinal neoplasia; it merely defers it. METHODS: Case of a patient with pouch polyposis was reviewed, and patient was prospectively studied after three months of sulindac therapy. RESULTS: Polyps not removed at first examination became much less prominent. Literature review reveals only one study of adenomas in pelvic pouches, with 7 cases of 38. CONCLUSION: Proctocolectomy and ileal pouch-anal anastomosis does not cure FAP, and multiple polyps can occur in the ileal pouch.
A captively maintained mature male opossum (Didelphis virginiana) utilized in a research protocol was presented with clinical signs of chronic diarrhea and severe muscle wasting. At necropsy, there was multifocal mural gastric, intestinal, and urinary bladder thickening, concurrent bilateral hydroureter and hydronephrosis, and extensive fibrous abdominal adhesions. Histologic evaluation revealed intestinal adenocarcinoma with coelomic metastasis to the stomach and urinary bladder. The adenocarcinoma was evaluated using histochemistry and electron microscopy. Paneth, enteroendocrine, and goblet cell differentiation was documented in primary and metastatic sites. This unique presentation of intestinal adenocarcinoma has not previously been reported in the opossum or any other animals. Intestinal neoplasia with Paneth cell differentiation is extremely rare and has been reported in humans with familial adenomatous polyposis.
Preservation of the rectum in chronic ulcerative colitis or familial polyposis conserves continence at the risk of recurrent disease or malignant change. Replacement of rectal mucosa with a graft of ileum in these benign colonic mucosal diseases conserves fecal continence without the threat of continuing disease or the development of carcinoma. Rectal mucosal replacement with construction of a rectal reservoir includes total colectomy, removal of the rectal mucosa-submucosa and its replacement with an ileal graft. A rectal reservoir is constructed when intestinal continuity is restored. Twenty-nine patients have undergone rectal mucosal replacement; 12 for familial polyposis and 17 for ulcerative colitis. Twenty-five patients have had intestinal continuity restored. Patients have been followed from three months to seven years after the restoration of intestinal continuity. Twenty-three patients have a satisfactory result. Fecal continence has been preserved. Patients pass an average of six stools in a 24 hour period.
We experienced a rare case of intestinal Behçet's disease simulating Crohn's colitis. A 70-yr-old female presented oral and genital ulcers, erythema nodosum, arthralgias, and abdominal pain. Regardless of our efforts, she died of septic shock. Autopsy showed punched-out ulcers in the terminal ileum and multiple longitudinal ulcers with inflammatory polyposis spreading from the ascending to the descending colon. The differential diagnosis of intestinal Behçet's disease versus Crohn's disease was difficult. However, microscopic findings showed nonspecific ulceration, and no evidence of Crohn's disease could be found. Clinically, the patient met the criteria of Behçet's disease, and punched-out ulcers in the ileocecal region, which is characteristic of intestinal Behçet's disease, confirmed the diagnosis of Behçet's colitis. Although rarely encountered, multiple longitudinal ulcers can involve all of the colon in Behçet's disease, like Crohn's disease.
Adenomatous polyposis coli (APC) is an important tumour suppressor in the mammalian intestinal epithelium. It binds to beta-catenin and its role as a tumour suppressor depends predominantly on its ability to downregulate soluble beta-catenin, a key effector of the Wnt signalling pathway. However, epithelial cells have a distinct subcellular pool of beta-catenin, or Drosophila Armadillo, which functions as a structural component of adherens junctions. Notably, APC proteins can be associated with these adherens junctions, and recent evidence points to a role for APC in cellular adhesion. Thus, APC--like beta-catenin/Armadillo--may have a dual role in Wnt signal transduction and in cellular adhesion, which could be relevant to its activity as a tumour suppressor.
An extremely rare case of primary gastrointestinal T-cell lymphoma involving the stomach and intestine is reported. Radiographic and endoscopic examinations showed multiple polypoid lesions covered by a normal-appearing mucosa in the stomach, duodenal bulb, and terminal ileum and numerous small aphthoid lesions throughout the entire colorectum. Histopathologic, immunohistochemical, and polymerase chain reaction studies were performed using paraffin-embedded or fresh-frozen specimens from endoscopic biopsies and endoscopic mucosal resections. All lesions were composed of small, atypical lymphoid cells, which were classified as low-grade pleomorphic lymphoma. The tumor cells expressed CD3, CD4, and the T-cell receptor gamma gene phenotype as well as human mucosal lymphocyte 1 antigen, suggesting that the lymphoma cells were derived from intraepithelial T lymphocytes. This is the first description of primary gastrointestinal T-cell lymphoma with expression of human mucosal lymphocyte 1 antigen and a novel morphology resembling multiple lymphomatous polyposis.
Small-intestinal malignancies are rare. Major risk factors for the development of these malignancies include celiac disease, which predisposes to both carcinoma and lymphoma. Crohn's disease patients have an increased risk of the development of adenocarcinoma, as do the inherited polyposis syndromes, FAP, and Peutz-Jehgers syndrome. Each of these conditions provides unique models for the development of malignancy.
PURPOSE: Patients treated with restorative proctocolectomy for familial adenomatous polyposis or ulcerative colitis occasionally develop disease in the ileal pouch similar to that originally present in the colon. We investigated the possibility of analogous involvement in the ileal pouch of juvenile polyposis patients. METHODS: Endoscopic surveillance for neoplasia throughout the gastrointestinal tract was performed, with retrieval of all polypectomy specimens for histologic classification using the criteria of Morson. RESULTS: Multiple large juvenile polyps were found in the ileal pouch of one patient less than 10 years after restorative proctocolectomy for hereditary juvenile polyposis. The pouch was much more severely affected than the proximal ileum, small intestine, or stomach. Although most polyps had a completely benign histologic appearance, three had moderate to severe dysplasia. DISCUSSION: Mucosal changes induced by bacteria or stasis of luminal contents may promote manifestation in the ileal pouch of the disease phenotype usually more evident in the colon. Patients with severe or generalized juvenile polyposis should be considered for periodic endoscopic surveillance of the ileal pouch beginning several years after restorative proctocolectomy.
Germ-line mutations of the APC gene are responsible for familial adenomatous polyposis (FAP), an autosomal dominantly inherited disease in humans. Patients with FAP develop multiple benign colorectal tumors. Recently, a mouse lineage that exhibits an autosomal dominantly inherited predisposition to multiple intestinal neoplasia (Min) was described. Linkage analysis showed that the murine homolog of the APC gene (mApc) was tightly linked to the Min locus. Sequence comparison of mApc between normal and Min-affected mice identified a nonsense mutation, which cosegregated with the Min phenotype. This mutation is analogous to those found in FAP kindreds and in sporadic colorectal cancers.