Carcinoma in an ileoanal pouch after restorative proctocolectomy for familial adenomatous polyposis.
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Adenomatous Polyposis Coli↗
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Mucin abnormality in colonic mucosa in patients with familial polyposis coli.
Colonic mucosa of the left colon in 41 patients with familial polyposis coli (FPC) was stained by a periodic acid-thionin Schiff/potassium hydroxide/periodic acid-Schiff method in which the normal colonic mucosa usually stained red and carcinoma stained blue or purple. In FPC 82.9% stained blue or purple whereas 35.5% stained blue or purple in normal controls. The data suggest that sialomucin properties of the flat mucosa surrounding polyps in FPC are different from those of the normal colon and that this simple technique may be useful for the early detection of high-risk family members in the FPC family.
Adenoma↗
Mucosal ischemia caused by desmoid tumors in patients with familial adenomatous polyposis: report of four cases.
PURPOSE: The study contained herein was undertaken to demonstrate that mesenteric desmoid tumors can cause significant symptoms other than by a mass effect and that surgery can be effective in treating these symptoms. METHODS: The medical records of patients with intra-abdominal desmoid disease in the David G. Jagelman Inherited Colorectal Cancer Registry were reviewed. Four cases are described in which intramesenteric desmoid tumors adjacent to the bowel caused mucosal ischemia in patients with familial adenomatous polyposis. RESULTS: In one patient with an ileorectal anastomosis, this was manifest by multiple small-bowel strictures and treated by multiple strictureplasties. The other three patients had ileal pouches and presented with bleeding and pain from mucosal ulceration. All pouches needed to be removed. CONCLUSION: Intra-abdominal desmoid tumors may cause problems other than by mass effect. Patients with familial adenomatous polyposis and symptoms suggestive of desmoid disease but with no detectable mass should not be denied surgery.
Adenomatous Polyposis Coli↗
New approach to surgical treatment of ulcerative colitis and polyposis coli without pelvic pouch. Experimental study in dogs.
A new method for the surgical treatment of ulcerative colitis and polyposis coli is described. Instead of preparing a pelvic pouch, the natural rectal pouch stripped of the diseased mucosa was used experimentally in dogs. The undisturbed muscular cuff of the rectum (12 cm from the anal verge) was covered by healthy vascularized mucosa of small bowel in such a manner that the dog could use its rectum as usual before surgery. The results are encouraging. The rectal reservoir is spared, with its sensitivity, continence and motor activity covered by healthy mucosa. The dogs thrived.
Adenomatous Polyposis Coli↗
Familial polyposis of the colon with subsequent development of a duodenal polyp: report of a case.
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Adenomatous Polyposis Coli↗
Initial experience of videocapsule endoscopy for diagnosing small-bowel tumors in patients with GI polyposis syndromes.
BACKGROUND: Small-bowel tumors frequently occur in familial adenomatous polyposis and other GI polyposis syndromes. These tumors are difficult to detect with conventional techniques. Our aim was to assess the utility of videocapsule endoscopy in the detection of small-bowel tumors in this setting. METHODS: We examined 19 familial adenomatous polyposis patients and 3 patients with either Peutz-Jeghers syndrome, hyperplastic polyposis, or Cowden disease. OBSERVATIONS: Prevalence of small-bowel polyps on videocapsule endoscopy was 59% in all patients, 52.6% in familial adenomatous polyposis patients, and 75% in a subgroup of familial adenomatous polyposis patients with exon 15 mutations. Videocapsule endoscopy was safe and well tolerated in all patients. CONCLUSIONS: Videocapsule endoscopy has a high yield in detecting small-bowel tumors in patients with GI polyposis syndromes. It may be especially indicated in familial adenomatous polyposis patients with the aggressive phenotype of the disease, e.g., mutations in exon 15.
Adenomatous Polyposis Coli↗
Cyclooxygenases and colon cancer.
There is considerable interest in the involvement of cyclooxygenase-2 (COX-2) in colon carcinogenesis and its progression, because nonsteroidal anti-inflammatory drugs (NSAIDs) reduce mortality from colon cancer and COX-2 is one of the known targets of NSAIDs. COX-2 mRNA and protein levels are increased in colon cancer tissues from patients and in some colon cancer cell lines. The relationship between COX-2 and colon cancer is further confirmed by studies using the murine models of adenomatous polyposis coli, in which NSAIDs and gene knockouts reduce the number of spontaneously developing intestinal polyps. COX-2 expression in intestinal epithelial cells increases resistance to apoptosis, promotes tumor angiogenesis, and enhances invasion and metastasis. COX-2 expression in stromal cells appears to have a role in tumor angiogenesis because tumor growth is attenuated when colon cancer cells are implanted in COX-2 knockout mice due to a decreased vascular supply to the tumors. Although NSAIDs act via COX-2 to inhibit colon cancer growth, there also appear to be COX-2 independent actions for NSAIDs. COX-2 selective inhibitors can be the core drugs for the prevention and the treatment of colon cancer.
Animals↗
Peutz-Jeghers syndrome: 78-year follow-up of the original family.
BACKGROUND: The association between heredity, gastrointestinal polyposis, and mucocutaneous pigmentation in Peutz-Jeghers syndrome (PJS) was first recognised in 1921 by Peutz in a Dutch family. This original family has now been followed-up for more than 78 years. We did mutation analysis in this family to test whether the recently identified LKB1 gene is indeed the PJS gene in this family. METHODS: The original family was retraced and the natural history of PJS was studied in six generations of this kindred by interview, physical examination, chart view, and histological review of tissue specimens. DNA-mutation analysis was done in all available descendants. FINDINGS: Clinical features in this family included gastrointestinal polyposis, mucocutaneous pigmentation, nasal polyposis, and rectal extrusion of polyps. Survival of affected family members was reduced by intestinal obstruction and by the development of malignant disease. A novel germline mutation in the LKB1 gene was found to cosegregate with the disease phenotype in the original family. The mutant LKB1 allele carried a T insertion at codon 66 in exon 1 resulting in frameshift and stop at codon 162 in exon 4. INTERPRETATION: The morbidity and mortality in this family suggest that PJS is not a benign disease. An inactivating germline mutation in the LKB1 gene is involved in the PJS phenotype in the original and oldest kindred known to be affected by PJS.
Adolescent↗
Use of transgenic mice in identifying chemopreventive agents.
Cancer chemoprevention uses natural- or synthetic chemical compounds to reverse, suppress or to prevent one or more of the biological events leading to the development of cancer. Chemopreventive agents are classified as blocking or suppressing according to their action on either the initiation or promotion-progression phases in experimental models using carcinogen treated animals. Transgenic animal technology has resulted in a plethora of murine models for cancer research providing insight into the complex oncogenic events contributing to the loss of cell cycle control and tumourigenesis. Transgenic models also offer an important opportunity to identify and study both tumourigens and chemopreventive agents. However, so far chemoprevention has in such models only been investigated to a limited degree and primarily in models with inactivated tumour suppressor genes. Studies show that spontaneous tumour developing due to loss of p53 function may be offset by preventive measures. The preventive actions of retinoids and polyamine synthesis inhibitors have been studied in the PIM mouse susceptible to lymphoma development. Most chemopreventive studies have been performed on murine familial adenomatous polyposis (FAP) models, which carry one non-functional apc gene and develop multiple intestinal adenomas upon inactivation of the wild type allele. Particularly non-steroidal anti-inflammatory drugs NSAIDs, which block COX-2, but also food components such as n-3 fatty acids show promising chemopreventive effects in these models. Transgenic cancer models demonstrate a strong gene-environment interaction, which is promising for the development of chemopreventive strategies.
Animals↗
Management of advanced duodenal polyposis in familial adenomatous polyposis.
Patients with familial adenomatous polyposis (FAP) are at increased risk for the development of periampullary cancer. The aim of this study was to evaluate the roles of endoscopic and surgical therapy in the management of advanced duodenal polyposis in FAP. From 1990 to 1995, seventy-four FAP patients were enrolled in a prospective endoscopic surveillance protocol. Among these, 11 (14.8%) developed advanced duodenal polyposis and one had duodenal adenocarcinoma. Six patients underwent endoscopic resection of duodenal (n=5) or ampullary adenomas (n=1). The following operations were performed in the remaining six patients: ampullectomy in four, open polypectomy in one, and a Whipple procedure in one. There was one patient who died of acute pancreatitis following endoscopic ampullectomy. The patient with invasive duodenal cancer died of local recurrence. Small polyps were observed at the site of previous resection in all (9 of 9) patients undergoing repeat endoscopy during a mean follow-up of 18 months (range 4 to 34 months). An endoscopic and local surgical resectional approach to advanced duodenal polyposis in FAP is fraught with high recurrence rates, although recurrent polyps are small and may be amenable to retreatment in the future. Long-term follow-up is necessary to prove that deaths from duodenal or ampullary cancer are prevented with this strategy.
Adenomatous Polyposis Coli↗
Surface structure of single-gland adenoma in familial adenomatous polyposis.
Magnifying videoendoscopic observation of a minute lesion with a single IIIL type cryptal orifice was reported. This minute lesion was a single-gland adenoma accompanied histopathologically by a bud of adenomatous gland and was located in the upper part of the mucosa. This finding regarding a pit pattern should make possible the endoscopic diagnosis of a colonic single-gland adenoma.
Adenomatous Polyposis Coli↗
Columnar cuff cancer after restorative proctocolectomy for familial adenomatous polyposis.
AIMS: Restorative proctocolectomy with ileoanal anastomosis is one of the treatments of choice for patients suffering from familial adenomatous polyposis (FAP). However, any residual rectal mucosa left behind after mucosectomy is at risk for the development of neoplasia. CASE REPORT: A 31 year old male patient with FAP underwent restorative proctocolectomy with a pelvic ileal pouch-anal anastomosis. Seven years later he presented with right inguinal and perianal pain. A diagnosis of invasive columnar cuff carcinoma was made. DISCUSSION: Islets of columnar epithelium may be left behind after restorative proctocolectomy, exposing the patient to later malignant change. This risk must be emphasised and prevented by regular surveillance of the anastomosis.
Adenocarcinoma↗
Ulex europaeus agglutinin-1 binding in hereditary bowel cancer.
Ulex europaeus agglutinin-1 (UEA-1) binds specifically to alpha-L-fucose, the terminal sugar of blood group substances H2 and Ley the expression of which is confined mainly to the proximal colon. The aim of the study was to determine whether there is neo-expression of alpha-L-fucose in the normal appearing mucosa of the distal bowel in subjects with or at risk of hereditary bowel cancer. UEA-1 binding was investigated by means of a direct lectin-peroxidase technique in tissues derived from patients with familial adenomatous polyposis (9) and affected (16) or at risk of (14) hereditary non-polyposis colorectal cancer. Control tissues were obtained from normal subjects and patients with sporadic bowel cancer and no family history of the condition. There was increased expression of alpha-L-fucose in some adenomas (mosaic pattern), in most carcinomas (diffuse), in hyperplastic polyps (crypt base), but not in normal appearing mucosa or transitional mucosa (adjacent to carcinoma) in any of the groups of patients examined. In view of the negative findings for normal-appearing mucosa, the suggestion that there is a pre-neoplastic field change in subjects at risk of hereditary bowel cancer may be unfounded.
Adenoma↗
Aberrant crypt foci as microscopic precursors of colorectal cancer.
Since the first detection of aberrant crypt foci (ACF) in carcinogen-treated mice, there have been numerous studies focusing on these microscopically visible lesions both in rodents and in humans. ACF have been generally accepted as precancerous lesions in regard to histopathological characteristics, biochemical and immunohistochemical alterations, and genetic and epigenetic alterations. ACF show variable histological features, ranging from hyperplasia to dysplasia. ACF in human colon are more frequently located in the distal parts than in the proximal parts, which is in accordance with those in colorectal cancer (CRC). The immunohistochemical expressions of carcinoembryonic antigen (CEA), beta-catenin, placental cadherin (P-cadherin), epithelial cadherin (E-cadherin), inducible nitric oxide synthase (iNOS), cyclooxygenase (COX-2), and P16INK4a are found to be altered. Genetic mutations of K-ras, APC and p53, and the epigenetic alterations of CpG island methylation of ACF have also been demonstrated. Genomic instabilities due to the defect of mismatch repair (MMR) system are detectable in ACF. Two hypotheses have been proposed. One is the "dysplasia ACF-adenoma-carcinoma sequence", the other is "heteroplastic ACF-adenoma-carcinoma sequence". The malignant potential of ACF, especially dysplastic ACF, makes it necessary to reveal the nature of these lesions, and to prevent CRC from the earliest possible stage. The technique of magnifying chromoscope makes it possible to detect "in vivo" ACF, which is beneficial to colon cancer research, identifying high-risk populations for CRC, and developing preventive procedures.
Adenomatous Polyposis Coli↗
[Long term surveillance of polyps and colorectal cancers].
It is necessary to assume the colorectal cancer follow-up after curative operation to detect cancer recurrence and new polyps or cancers. A good follow-up by endoscopy is also necessary for patients with colorectal polyps and for patients with familial adenomatous polyposis.
Adenomatous Polyposis Coli↗
[Transsacral fistula of an ileal pouch-anal anastomosis].
For patients with ulcerative colitis and familial adenomatous polyposis the restorative proctocolectomy with ileo-anal-pouch anastomosis is the surgical treatment of choice. Leakage from the ileo-pouch anastomosis is the surgical treatment of choice. Leakage from the ileo-pouch-anastomosis can be a difficult to manage complication, which in some cases resists all attempts at local repair. A surprising complication of a 28 years old woman patient with an ileo-anal-pouch anastomotic fistula is presented. The fistula developed the 4th day postoperatively. Local irrigation and transanal drainage seemed to have a good result, the patient being examined after two weeks. During an apparently better evolution, after one month, the patient developed a transsacral fistula with local abscess and osteolysis. The ileo-anal-pouch anastomosis was converted to a less comfortable conventional ileostomy, but with good local and general final result.
Adenomatous Polyposis Coli↗
Bottom-up histogenesis of colorectal adenomas: origin in the monocryptal adenoma and initial expansion by crypt fission.
The adenoma:carcinoma sequence is well established. Understanding the molecular pathology of the adenoma is therefore important. There is great controversy within the field. The Vogelstein group champions the "top-down" theory (colorectal adenomas arise and grow across the mucosal surface and down into the crypts), whereas other studies, including our own, propose "bottom-up" spread. Serial sections of 40 small (<3 mm) sporadic colorectal adenomas were stained with H&E, MIB-1, and for beta-catenin. 10 early adenomas were Feulgen-stained and microdissected. We also examined the flat mucosa of three patients who had undergone colectomies for familial adenomatous polyposis (FAP) and specimens from a XO/XY individual with FAP, the latter using in situ hybridization for the Y chromosome. In the earliest sporadic adenomas, there were crypts entirely filled with adenomatous epithelium, which showed proliferative activity and nuclear localization of beta-catenin. There was a sharp cutoff between crypt epithelial cells showing nuclear beta-catenin and surface cells with membrane staining. In slightly larger lesions, adenomatous spread from above was seen. Microdissected adenomas showed multiple fission events, with proliferation distributed equally throughout. In FAP tissue, numerous isolated monocryptal adenomas, which were clonal in origin, were seen. Examination of adenomas in the XO/XY individual showed no instances of XY or XO adenomatous epithelium growing down into crypts of the other genotype. Both sporadic and FAP adenomas start as a unicryptal adenomas and grow initially by crypt fission--a bottom-up pattern. Later, in sporadic adenomas, there is evidence of growth down into adjacent crypts (top-down).
Adenoma↗
[Siblings of familial adenomatosis coli with gastric cancer--case report].
Adenomatosis coli is recently regarded as a systemic disease with a predisposition to multiple tumor formation. We report siblings of familial adenomatosis coli with gastric cancers. Case 1 was a 58 year-old elder brother. His diagnosis was familial adenomatosis coli accompanied with colon cancer and simultaneous early gastric cancer. Total colectomy and partial gastrectomy were carried out on Mar. 13, 1984 at our hospital. Numerous polyps over the whole colon and an ulcerative tumor in the hepatic flexure were found in the resected colon. Histologically tubular adenocarcinoma were demonstrated in the ulcerative tumor, and all other polyps were adenomas. In the resected gastric specimen, there were two shallow, depressed lesions on the each anterior and posterior wall of the antrum. Histologically both of them were adenocarcinoma confined within the mucosa. Postoperative course was satisfactory and he is quite healthy 2 and a half years after surgery. Case 2 was a 56 year-old younger brother. He received a partial gastrectomy for advanced gastric cancer at another hospital on May 20, 1982. In one and a half year from the surgery, a large lung tumor (probably metastasis of the gastric cancer) was found and he received chemotherapy. He also received radiation therapy in June, 1984 and during this admission barium enema study was performed. It revealed numerous polyps over the whole colon. No cancerous lesions were found. He died of lung tumor on Dec. 8, 1985. The similar siblings were first reported by Kokaji et al. in 1984, and our cases seem to be the second ones.
Adenocarcinoma↗