1/26. Coexisting carcinoid tumors in familial adenomatous polyposis-associated upper intestinal adenomas.Upper gastrointestinal polyps and extraintestinal tumors are well recognized in association with familial adenomatous polyposis (FAP). Although carcinoid tumors have been reported in association with sporadic colonic neoplasms and ulcerative colitis, to date, carcinoids have not been reported in association with FAP. We report a patient with FAP who has recurrent carcinoid tumors located at the bases of duodenal adenomas. The genetic basis of carcinoid neoplasms is still uncertain. This report may represent the clinical effect of the APC gene mutation on the enterochromaffin cell line manifesting as recurrent carcinoid tumors in physical association with intestinal adenomas. Future genetic analysis and epidemiological studies may be of value in determining whether a true association exists.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
2/26. Cribriform-morular variant of papillary carcinoma: a distinctive variant representing the sporadic counterpart of familial adenomatous polyposis-associated thyroid carcinoma?We describe herein four cases of an unusual sporadic thyroid tumor that shares the morphologic features of a distinctive follicular cell neoplasm previously proposed as a feasible indicator of familial adenomatous polyposis. We also review five other similar cases reported in the literature. All of the nine patients were young women, aged 16 to 30 years. Grossly, the neoplasms measured 1.5 to 5.6 cm; they were solid and solitary, except one case, which showed two tumor nodules, one in each lobe. Histologically, the lesions were encapsulated, and they exhibited an intricate blending of cribriform, follicular, papillary, trabecular, and solid patterns of growth, with morular (squamoid) areas. Cribriform structures were prominent, being formed by anastomosing bars and arches of cells in the absence of intervening fibrovascular stroma. Follicular areas were usually devoid of colloid, and the papillae were lined by columnar cells. There were focal areas of trabecular arrangement reminiscent of hyalinizing trabecular adenoma. The tumor cells were cuboidal or tall, with frequent nuclear pseudostratification and abundant eosinophilic-to-oxyphilic cytoplasm. The nuclei were usually hyperchromatic, but nuclear grooves, pale or clear nuclei, and intranuclear cytoplasmic inclusions were variably present. Morules with peculiar nuclear clearing caused by biotin accumulation were scattered in the tumors. Vascular and/or capsular invasion were noted in all of the cases except one, and lymph node metastasis was found in two cases. Immunohistochemical stains showed reactivity for thyroglobulin, epithelial membrane antigen, cytokeratins (including 34betaE12), vimentin, estrogen and progesterone receptors, bcl-2, and Rb proteins. Follow-up in seven cases showed that all of the patients were alive with no evidence of disease at 1 to 13 years after diagnosis. Thus, the behavior of this variant seems to be similar to that of conventional papillary carcinoma Because of the distinctive histologic features, we propose naming this tumor the cribriform-morular variant of papillary carcinoma.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
3/26. A case of myoepithelial carcinoma displaying biallelic inactivation of the tumour suppressor gene APC in a patient with familial adenomatous polyposis.Familial adenomatous polyposis (FAP) is an autosomal dominant disorder caused by mutation of the APC gene. It is characterised by the appearance of hundreds to thousands of colorectal adenomas in adolescence and the subsequent development of colorectal cancer. Various extracolonic malignancies are associated with FAP, including desmoids and neoplasms of the stomach, duodenum, pancreas, liver, and brain. We present a family affected by FAP with an exon 14 APC mutation displaying two rare extracolonic lesions, a hepatoblastoma and a myoepithelial carcinoma. The hepatoblastoma was found in a male patient aged 2 years. The second lesion, a myoepithelial carcinoma of the right cheek, was found in a female patient aged 14 years. Inactivation of the normal APC allele was demonstrated in this lesion by loss of heterozygosity analysis, thus implicating APC in the initiation or progression of this neoplasm. This is the first reported case of this lesion in a family affected by FAP.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
4/26. Cribriform-morular variant of papillary carcinoma: the sporadic counterpart of familial adenomatous polyposis-associated thyroid carcinoma. A case report with clinical and molecular genetic correlation.The increased incidence of thyroid carcinomas in familial adenomatous polyposis (FAP) patients is well recognised. These thyroid neoplasms display distinctive clinicopathological features and generally show good prognostic outcome. Recently, unusual sporadic tumours that share the morphological features of FAP-associated thyroid carcinomas have also been described. In this report, we document a case of a thyroid tumour in a previously well, 46-year-old female. histology revealed a circumscribed neoplasm composed of tubular, papillary, cribriform and solid areas. The pseudostratified columnar tumour cells showed occasional nuclear grooves and rare nuclear inclusions. immunohistochemistry showed positive staining with antibodies to cytokeratin AE1/AE3, oestrogen and progesterone receptor proteins. Focal immunoreactivity was also noted with antibodies to thyroglobulin, epithelial membrane antigen, 34betaE12 and cytokeratin CK7. The absence of polyps on colonoscopy and germline mutation in the adenomatous polyposis coli (APC) gene provides evidence that this tumour represents the sporadic counterpart of FAP-associated thyroid carcinoma. The patient is well with no evidence of disease 7 months following resection of the tumour. The differential diagnoses and molecular genetics of this unusual tumour are discussed.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
5/26. Giant desmoid tumor of the abdominal wall associated with familial adenomatous polyposis.In this paper a case of vast desmoid tumor of the abdominal wall associated with familial adenomatous polyposis is reported. Desmoid tumors represent a particular type of fibrous neoplasms with a higher prevalence in females. They are extremely rare in the sporadic form, while they are found in about 10% of patients affected by familial adenomatous polyposis. Despite their benign histology and the absence of metastatic potential, they can be considered as fibrosarcomas with a low level of malignancy because of their locally invasive nature. The treatment of choice is surgical excision, as wide as possible, aimed at preventing recurrences, which are frequent in this tumor type. The usefulness of complementary therapies such as radiotherapy, hormone or chemotherapy, is not entirely clear.- - - - - - - - - - ranking = 0.5keywords = neoplasm (Clic here for more details about this article) |
6/26. risk of gastric cancer among Korean familial adenomatous polyposis patients. Report of three cases.Gastric cancer has been recognized as an extracolonic manifestation in patients with familial adenomatous polyposis (FAP). In korea, gastric cancer is the most common malignant neoplasm. In a recent survey, we collected data from 72 Korean patients with FAP. Among them, three (4.2 percent) were found to have associated gastric cancer. This incidence of gastric cancer in our series is much higher than the previous reports from japan and other countries. The expected cumulative incidence of gastric cancer among these 72 patients was 0.44, which gives the standardized incidence ratio of 6.9 (95 percent CI, 1.4-20.1). This difference in incidence of gastric cancer was statistically significant (P less than 0.05), which implies that patients with FAP are at significantly higher risk of developing gastric cancer compared with the general population in korea. These findings confirm an increased risk of gastric cancer in FAP patients, even in a region where gastric cancer is highly prevalent.- - - - - - - - - - ranking = 0.5keywords = neoplasm (Clic here for more details about this article) |
7/26. Loss of normal allele of the APC gene in an adrenocortical carcinoma from a patient with familial adenomatous polyposis.Endocrine neoplasms have been reported occasionally in patients with familial adenomatous polyposis (FAP). An adrenocorotical carcinoma was studied in a patient with a family history of FAP. loss of heterozygosity (LOH) in the region close to the adenomatous polyposis coli (APC) gene was detected in this carcinoma, and evidence was obtained that there was a loss of the normal allele of the APC gene. This is the first demonstration of LOH at the APC locus in adrenocortical tumors. The present results and our previous data on LOH in a recurring desmoid tumor suggest that the heterozygous mutant/wild-type condition of the APC gene may give rise to benign tumors, and that functional loss of this gene leads to development of tumors not only in the colon but also in other various parts of the body in FAP patients.- - - - - - - - - - ranking = 0.5keywords = neoplasm (Clic here for more details about this article) |
8/26. Serrated adenoma of the duodenum.The duodenum is the most common site for extracolonic adenomas in patients with familial adenomatous polyposis (FAP). These adenomas are usually tubular, villous, or tubulovillous. This report describes a patient with FAP who had a duodenal adenoma with a different histology--it had the microscopic attributes of a serrated adenoma--tumours that are relatively common in the colorectal mucosa and are occasionally found in the stomach. A 78 year old man with FAP and multiple adenomas was colectomised and the rectum amputated. Several years later he developed silent jaundice. The surgical specimen showed an adenomatous growth juxtaposing the papilla of Vater. The adenoma had epithelial fronds with crenated, sawtooth-like configurations caused by scalloped epithelial infolding. The nuclei covering the notched fronds were pleomorphic, stratified, either cigar shaped with irregular chromatin deposits or vesicular shaped with a large nucleolus. One area showed involvement of a duct by neoplastic epithelium with extension into periluminal glands. No invasive carcinoma was present. Serrated adenomas differ from tubular and villous adenomas in their histological organisation and their initial pattern of cell proliferation and genotypic aberration. Increased awareness of the existence of serrated neoplasms in the duodenum may result in similar cases being reported in the future.- - - - - - - - - - ranking = 0.5keywords = neoplasm (Clic here for more details about this article) |
9/26. Intraductal papillary mucinous neoplasm of the pancreas in a patient with attenuated familial adenomatous polyposis.A 67 year old man with a clinical diagnosis of attenuated familial adenomatous polyposis (AFAP) and a past history of synchronous colon cancers in the transverse colon was also found to have an intraductal papillary mucinous neoplasm (IPMN) of the pancreas. In addition, several foci of heterotopic gastric oxyntic mucosa were noted in the duodenum, interspersed with flat and polypoid adenomas. The duodenal adenomas showed low grade dysplasia, loss of adenomatous polyposis coli (APC) protein expression, but retention of beta catenin staining, localised to the nucleus and cytoplasm. The IPMN in the pancreas showed an identical immunohistochemical profile to the duodenal adenomas. The heterotopic gastric foci in the duodenum were negative for the APC protein, and beta catenin staining was membranous in location. Although the patient did not show germline truncating APC mutations or mutations in the MYH gene, the past history, clinical features, and immunohistochemical profile of the various lesions suggest strongly that the IPMN is part of the spectrum of lesions encountered in AFAP. Whether the heterotopic oxyntic gastric mucosa in the duodenum is also related is unclear, but it may represent a forme fruste of fundic gland polyps.- - - - - - - - - - ranking = 2.5keywords = neoplasm (Clic here for more details about this article) |
10/26. Duodenal cancer arising from the remaining duodenum after pylorus-preserving pancreatoduodenectomy for ampullary cancer in familial adenomatous polyposis.We herein report a rare occurrence of duodenal cancer arising from the remaining duodenum after pylorus-preserving pancreatoduodenectomy for ampullary cancer in familial adenomatous polyposis (FAP). In this patient, proctocolectomy and ileoanal anastomosis for FAP had been performed 11 years earlier. During the current admission, the patient was diagnosed with adenocarcinoma in the Vater's ampulla using imaging and pathological examinations. In addition, a pylorus-preserving pancreatoduodenectomy was performed. The tumor was a well-differentiated tubular adenocarcinoma and no other polyps were identified in the duodenum by pathological examination. However, 1 year after surgery, a polypoid lesion measuring 15 x 15 mm was indicated in the remaining duodenum by endoscopic surveillance. This lesion was completely resected by endoscopic mucosal resection and the resected specimen revealed well-differentiated tubular adenocarcinoma in an adenomatous lesion. This report suggests that resection of the total duodenum is essential for duodenal neoplasms in FAP to prevent a recurrence in the remaining duodenum.- - - - - - - - - - ranking = 0.5keywords = neoplasm (Clic here for more details about this article) |
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