1/34. Cronkhite-canada syndrome associated with advanced rectal cancer treated by a subtotal colectomy: report of a case.A 41-year-old man with Cronkhite-canada syndrome presented with multiple juvenile polyps with hyperplastic and adenomatous changes throughout his stomach and entire colorectum. dysgeusia was recognized and the degree of hypoproteinemia was remarkable. A barium enema study and colonofiberscopy also revealed an advanced cancer in the rectum. Chronic hepatitis B and membranous glomerulonephritis were also present. It was difficult to design a conservative protocol using steroids for the treatment of protein-loosing enteropathy because the patient was a hepatitis b virus carrier. As a result, a subtotal colectomy while preserving the cecum with cecorectal anastomosis was performed. Pathologically, the ulcerated rectal tumor was a moderately differentiated adenocarcinoma with invasion into the muscularis propria. Most polyps showed cystically dilated glands without dysplasia or edematous stroma with inflammatory cell infiltration. A few polyps were juvenile-type polyps with adenoma components. Although no remarkable improvement was observed in the hypoproteinemia postoperatively, an alpha1-antitrypsin clearance test showed a significant decrease in protein loss from the gastrointestinal tract, which was only about one third of the loss seen preoperatively. These findings lead us to conclude that when improvement using conservative treatment can be neither obtained nor is expected, then the use of surgery should be considered when treating patients with Cronkhite-canada syndrome.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
2/34. Feminizing sertoli cell tumor associated with peutz-jeghers syndrome.peutz-jeghers syndrome (PJS) is an autosomal dominant disorder characterized by the association of mucocutaneous pigmentation and multiple gastrointestinal hamartomatous polyps and with an increased risk of developing gonadal sex tumors besides other malignancies. We describe a 7 1/2 year-old boy with PJS and bilateral gynecomastia. He has had buccal pigmentation since 1.5 years and had been operated for rectal polyp excision at 3.5 years. On physical examination, his height was at the 90th percentile, and his height age and bone age were 9 and 10 1/2 years, respectively. Increased melanotic buccal pigmentation of the lips and bilateral gynecomastia were noticed. Both of the testes were firm, non-tender and smooth on the surface, and each measured 8 ml. Hormonal measurements were all in the prepubertal range. testis ultrasonography showed bilateral hyperechogenic areas within the glands. When he was operated for invagination and an ileum segment full of polyps was resected, bilateral testicular biopsies were also performed. Histopathological evaluation of the testes revealed bilateral multicentric benign Sertoli cell tumors. The aromatase inhibitor testolactone was started to slow skeletal maturation. On the basis of this and previous reports, PJS associated with sex-cord tumors is increasingly recognized in males as well as in females.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
3/34. Gastric-type adenocarcinoma of the duodenal second portion histogenetically associated with hyperplasia and gastric-foveolar metaplasia of Brunner's glands.We report a case of a pedunculated polyp with a focus of gastric-type adenocarcinoma arising in the opposite side of the papilla Vater of the duodenal second portion. The carcinoma was surrounded by lobules of hyperplastic Brunner's glands. Immunohistochemically the carcinoma tissue showed both gastric foveolar-type mucin ( MUC5AC) and pyloric/Brunner's gland-type mucin ( MUC6), in which proliferating cells positive for MIB-1 (Ki-67) were scattered diffusely. Most of the hyperplastic Brunner's glands were positive for MUC6, while cells toward the lumen in the superficial layer were positive for MUC5AC and MIB-1. This directional pattern of differentiation of Brunner's glands has recently been demonstrated by our group in the histogenetic course of gastric metaplasia originating directly from Brunner's glands. Therefore the present carcinoma is thought to have developed under induction of gastric-foveolar differentiation in a manner very similar to that of gastric metaplasia in hyperplastic Brunner's glands.- - - - - - - - - - ranking = 10keywords = gland (Clic here for more details about this article) |
4/34. Two autopsy cases of diffuse gastrointestinal polyposis with ectodermal changes. Cronkhite-canada syndrome.Two autopsy cases of Cronkhite-canada syndrome were reported. The caused of hypoproteinemia, electrolyte imbalance and ectodermal changes were discussed with reference to previously reported cases. The mechanism of protein loss was probably due to outflow into the intestinal lumen of the mucous substance in the cystically dilated glands, directly and/or indirectly followed by loss of mucosal surface. Electrolyte imbalance probably developed from gastrointestinal loss as well as poor substitution. The ectodermal changes were probably not a subsequent part of the emaciation or hypoproteinemis, but an inherent part of this disease. Therapy, whether substitution or surgical procedure, should be selected in order to control the general condition of the patient.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
5/34. Duodenal polypectomy of Brunner's gland hyperplasia using a novel laparoscopic technique. A case report.Brunner's gland hyperplasia rarely is associated with clinical problems, and it accounts for only about 10% of benign tumors of duodenum. Therapeutic intervention is indicated when Brunner's gland hyperplasia evokes symptoms such as indigestion, bleeding, or obstruction. Endoscopic intervention (esophagogastroduodenoscopy) often can be used effectively, and in some cases may be preferable because of its minimal invasivencss. However, when the lesion is too large to pass through the endoscopic snare, endoscopic treatment is not possible. In these cases, a laparoscopic procedure may provide a novel approach to resection of the polyp, while still serving as a minimally invasive approach. We report the case of a 28-year-old woman with a large duodenal polyp treated by laparoscopic polypectomy.- - - - - - - - - - ranking = 6keywords = gland (Clic here for more details about this article) |
6/34. Severe intestinal bleeding caused by intestinal metastases of a primary angiosarcome of the thyroid gland.A 75 year old male presented with gastrointestinal bleeding after resection of both upper lobes of the lungs because of metastases. One year ago an angiosarcoma was the reason for a complete removal of the thyroid gland. In esophago-gastro-duodenoscopy we found multiple hemorrhagically stained polyploids lesions in the postbulbar duodenum and jejunum. colonoscopy showed isolated polyploid lesions of the right flexura. Because of persistent gastrointestinal bleeding a diagnostic laparotomy was done. Intraoperative intestinoscopy demonstrated multiple bleeding metastasis. To remove many of the bleeding lesions two longer intestinal segments of the jejunum and ileum were resected. The histology of the metastases showed arrangements of polygonal cells with prominent nucleoli and atypical mitosis. immunohistochemistry identified CD 31, vimentin and factor viii associated antigen. There was an erosion of the superficial intestinal mucosal cells with resulting hemorrhage; same histology had been found in the thyroid gland and the right upper lobe of lung. Eight days after surgery the patient died because of respiratory and circulatory insufficiency.- - - - - - - - - - ranking = 6keywords = gland (Clic here for more details about this article) |
7/34. adenocarcinoma and multiple adenomas of the large intestine, associated with Cronkhite-canada syndrome.The Cronkhite-canada syndrome is a rare non-hereditary disorder with generalised gastrointestinal polyposis, associated with ectodermal changes. We report here a case of adenocarcinoma and multiple adenomas of the large intestine associated with Cronkhite-canada syndrome in a 61-year-old Japanese man. Histologically, the rectal tumour was composed of well-differentiated tubular adenocarcinoma, admixed with foci of adenomatous components, and associated with hyperplastic mucosa of Cronkhite-canada syndrome. Multiple polyps, >20 polyps < or = 2.0 cm in diameter, were found near the carcinoma of the resected rectum. Histologically, superficial parts of the polyps were composed of tubular adenomas, and basal parts of the polyps were hyperplastic dilated glands. It was speculated that, in the present case, the rectal adenocarcinoma arose from mucosal hyperplasia (Cronkhite-canada polyp)-adenoma-carcinoma pathway. This suggested that Cronkhite-canada syndrome has definite malignant potential, although the frequency of malignant transformation is thought to be low in Cronkhite-canada syndrome.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
8/34. Vanek's tumor (inflammatory fibroid polyp). Report of 18 cases and comparison with three cases of original Vanek's series.Eighteen cases of Vanek's tumors are presented. The patients included nine men and nine women between the ages of 45 and 93 years (mean, 66.2 years). Nine cases were clinically diagnosed as polyps of the gastric antrum, five cases as polyps of the stomach (not otherwise specified), one polyp was located in the ileum and the three remaining polyps in the small intestine (not otherwise specified). The thirteen polyps with available size information measured from 0.4 to 5 cm in the greatest diameter (mean, 2.2 cm). Immunohistochemically, the affections were positive for vimentin (18/18) and CD34 (15/18). All the cases negative for CD34 also lacked concentric onion skin-like formations of the spindle cells around glands and vessels. The different immunophenotype and absence of concentric formations could be explained by the existence of two different lesions commonly designated as Vanek's tumor (inflammatory fibroid polyp) or by the hypothesis of various evolutional stages. In the differential diagnosis, it is important to distinguish namely eosinophilic gastroenteritis, gastrointestinal stromal tumor, inflammatory pseudotumor, hemangioendothelioma, and hemangiopericytoma. In contrast to gastrointestinal stromal tumors, genetically no substitution, deletion, or insertion occurred in c-kit exon 11 in all analyzed samples. Likewise, no deletion or insertion in part of c-kit exon 9 was observed.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
9/34. adenoma with gastric differentiation (so-called pyloric gland adenoma) in a heterotopic gastric corpus mucosa in the rectum.In a 46-year-old man, a pedunculated rectal polyp measuring 3.0x3.0x2.0 cm was diagnosed histologically as a pyloric gland-type adenoma arising in heterotopic gastric corpus mucosa. The luminal site was covered by glands of the gastric foveolar type, displaying focal marked proliferation interpreted as low-grade intraepithelial neoplasia. A bidirectional gastric differentiation was found: most lower glandular structures showed positivity for the deep gastric mucin core protein Muc 6 and superficial positivity for gastric foveolar epithelium mucin core protein Muc 5AC. Pyloric gland adenoma has so far been described in one larger series only and a few case reports of the stomach, gallbladder, pancreatic duct and within heterotopic gastric corpus mucosa of the duodenal bulb. The present case report is the first case of a pyloric gland-type adenoma within a gastric corpus heterotopia of the rectal mucosa.- - - - - - - - - - ranking = 9keywords = gland (Clic here for more details about this article) |
10/34. Multicentric gastrointestinal and extraintestinal leiomyosarcomatosis: a case report.This article presents a case of synchronous polypoid primary adenocarcinoma of the transverse colon without lymph node or distance metastasis. During the postmortem examination, polypoid smooth muscle tumors in the patient's gastrointestinal tract and extensive extraintestinal smooth muscle tumors in the patient's liver, lungs, and visceral organs, including the diaphragm, parathyroid gland, bone marrow of the vertebrae, and subcutaneous tissue of the left wrist, were reviewed to determine their origin. Histomorphologically, all of the tumors were classified as leiomyosarcomas.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
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