1/34. Autoaggressive inflammation of the myenteric plexus resulting in intestinal pseudoobstruction.After a 3-year history of severe constipation, a 16-year-old girl required surgery to be relieved of impacted stools. Histologic examination showed ganglionitis in the myenteric plexus of the large bowel and ileum, whereas the submucosal plexus was spared. At this time, antineuronal nuclear antibodies (ANNA-1, anti-Hu) were found at high titer in the serum of the patient. One and a half years earlier, a paravertebral ganglioneuroblastoma had been removed. Histologic examination had shown undifferentiated neuroblasts and morphologically mature ganglion cells with both cell types embedded in an inflammatory infiltrate morphologically similar to the lymphoplasmocytic infiltration seen in the myenteric plexus. The patient's serum was found to bind to nuclei of mouse intestinal tract neurons, thus fulfilling defining criteria for ANNA-1. The serum also reacted with antigens of defined molecular weight in a Western blot, thus fulfilling defining criteria for anti-Hu. Expression of the Huantigen could be visualized in the nuclei of the patient's tumor cells by immunohistochemistry. These tests showed that an antitumor inflammatory response was the cause of the bowel disease. This is the first report of a tumor from the neuroblastoma group that caused paraneoplastic intestinal pseudoobstruction. Ganglionitis and subsequent aganglionosis are the hallmark of the morphologic diagnosis which cannot be obtained by suction biopsy in patients with intact submucosal plexus. Instead, serum testing for autoantibodies can reveal the etiology.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
2/34. Refractory sprue syndrome with clonal intraepithelial lymphocytes evolving into overt enteropathy-type intestinal T-cell lymphoma.INTRODUCTION: Recently, patients with refractory sprue have been shown to contain a clonal proliferation of phenotypically abnormal intraepithelial lymphocytes in their intestine. Whether this signifies early enteropathy-type intestinal T-cell lymphoma (EITCL) or a reactive condition is not clear. We report on a patient presenting with the findings of refractory sprue who subsequently developed overt EITCL. MATERIAL AND methods: Duodenal biopsies from 1997 (refractory sprue) and duodenal and jejunal biopsies from 1998 (intestinal T-cell lymphoma) were compared by immunohistochemistry and PCR for the detection of T-cell receptor (TCR)-gamma gene rearrangements. Clonal PCR products were sequenced. RESULTS: The duodenal biopsies from both 1997 and 1998 and the jejunal tumor biopsy showed villus atrophy and an increase of intraepithelial lymphocytes with an abnormal immunophenotype (CD3 , CD4-, CD8- and TCR-beta-). In all duodenal specimens including the one from 1997, and the jenunal tumor biopsy, an identical clonal amplificate was detected by enzymatic amplification of the TCR-gamma gene. CONCLUSION: These data suggest that refractory sprue containing a clonal proliferation of phenotypically abnormal intraepithelial lymphocytes may represent an early manifestation of EITCL. The detection of immunohistochemical negativity for several antigens normally found on intraepithelial lymphocytes such as CD8 or the TCR-beta chain in combination with clonal T-cell populations by PCR may be helpful in identifying refractory sprue with a malignant transformation.- - - - - - - - - - ranking = 0.5keywords = antigen (Clic here for more details about this article) |
3/34. Multifocal epithelioid angiosarcoma of the small intestine.A 67-year-old man presented with weight loss, intermittent severe abdominal pain and melaena. Initial radiology (including abdominal ultrasonography), gastroscopy and colonoscopy did not demonstrate any lesions that could explain the complaints. Three weeks later, upper gastrointestinal and small-bowel barium studies revealed two areas in the small intestine with an abnormal mucosal pattern. Explorative laparotomy revealed three tumoral lesions. Three partial enterectomies were performed. Gross examination showed centrally depressed dark reddish tumoral lesions extending from the mucosa throughout the full thickness of the bowel wall (diameter varying between 1.6 cm and 2.2 cm). The tumours, composed of large, plump, polygonal cells showing little architectural differentiation, were mainly situated in submucosa and muscularis propria. The growth pattern appeared rather solid. The epithelioid cells showed pronounced nuclear pleomorphism and atypia with central large nucleoli. There were several small blood vessels with occasional anaplastic endothelial cells. Immunohistochemical staining demonstrated an intense expression of CD 31, CD 34, factor viii related antigen and keratin. This supported the diagnosis of an epithelioid angiosarcoma. The patient died 3 months after diagnosis. Tumours of the small intestine are very rare, and angiosarcomas of the small intestine are even more rare. Epithelioid variants have only been described in two patients and only one of these had a multifocal presentation. The prognosis is very poor. Because of the epithelioid growth pattern and the cytokeratin expression, these tumours may erroneously be diagnosed as a carcinoma.- - - - - - - - - - ranking = 0.5keywords = antigen (Clic here for more details about this article) |
4/34. Primary vaginal adenocarcinoma of intestinal type arising from an adenoma: case report and review of the literature.A 1 cm polypoid lesion was encountered on the posterior vaginal wall in a 56-year-old woman with no history of diethylstilbestrol exposure that on microscopic examination was a moderately differentiated adenocarcinoma of intestinal type. The tumor was cytokeratin 20 and carcinoembryonic antigen positive and negative for cytokeratin 7. Mucin histochemistry demonstrated the presence of o-acetylated sialomucin, a specific marker of large intestinal differentiation. The initial interpretation favored a metastasis from a colonic adenocarcinoma, but clinical investigations showed no evidence of a primary gastrointestinal lesion. The morphology, histochemical, and differential cytokeratin profile led to the lesion being reinterpreted as a primary intestinal-type adenocarcinoma of the vagina arising from a tubular adenoma. Although a very rare tumor, awareness of this lesion is important as it must be distinguished from metastatic adenocarcinomas from other sites.- - - - - - - - - - ranking = 0.5keywords = antigen (Clic here for more details about this article) |
5/34. adenocarcinoma of the small intestine arising in Crohn's disease. Demonstration of a tumor-associated antigen in invasive and intraepithelial components.A segment of small intestine surgically removed from a man with intestinal obstruction was found to have coexisting regional enteritis and an invasive adenocarcinoma associated with an area of intraepithelial neoplasia. The cells of the adenocarcinoma and the intraepithelial neoplasia contained a tumor-associated surface antigen capable of reacting with an antiserum prepared against a colonic carcinoma. The importance of thorough sampling of specimens of regional enteritis in accurate reporting of the incidence of carcinoma is stressed, as is the use of immunohistologic techniques as an adjunct to the morphologic diagnosis of preinvasive neoplasia.- - - - - - - - - - ranking = 2.5keywords = antigen (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 = 0.5keywords = antigen (Clic here for more details about this article) |
7/34. Immediate haemolytic transfusion reaction due to anti-Inb.An immediate haemolytic transfusion reaction was investigated in a patient with intestinal cancer. The causative antibody was directed against a high-frequency antigen Inb that was presumably produced as a result of pregnancies. This is the first case of a severe transfusion reaction due to this alloantibody.- - - - - - - - - - ranking = 0.5keywords = antigen (Clic here for more details about this article) |
8/34. Extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma) arising in the small intestine with monoclonal cryoglobulinemia.A case of small intestinal extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma) with monoclonal cryoglobulinemia is described. The patient was a woman in her mid-sixties with purpura of the bilateral lower legs and abdominal pain. An immunoserological investigation showed expression of IgM-kappa type monoclonal cryoglobulin. A renal biopsy specimen revealed proliferative glomerulonephritis with cryoglobulin deposition. physical examination disclosed a stenosis, edematous changes and ascariasis in the small intestine. In aspiration cytology of the ascites, proliferation of the atypical lymphoid cells with plasmacytoid differentiation was observed. These cells were positive for B-lineage antigens in immunocytochemistry, and showed an immunoglobulin heavy-chain gene rearrangement in Southern blotting and chromosomal alteration in G-banded karyotype analysis. Although medicinal treatment was used, the patient died of general prostration. The diagnosis of intestinal MALT lymphoma was made at autopsy. Expression of API2-MALT1 fusion transcripts was detected by reverse transcription-polymerase chain reaction analysis using formalin-fixed, paraffin-embedded tissue. Intestinal MALT lymphomas with API2-MALT1 expression have distinctive forms of infiltration compared with those without translocation. Therefore, detection of API2-MALT1 fusion transcripts is useful for evaluating the prognosis and clinical behavior of the disease.- - - - - - - - - - ranking = 0.5keywords = antigen (Clic here for more details about this article) |
9/34. Multiple metastases to the small bowel from large cell bronchial carcinomas.AIM: Metastases from lung cancer to gastrointestinal tract are not rare at postmortem studies but the development of clinically significant symptoms from the gastrointestinal metastases is very unusual. methods: Formalin-fixed, paraffin-embedded tissues were cut into 5 microm thick sections and routinely stained with hematoxylin and eosin. Some slides were also stained with Alcian-PAS. Antibodies used were primary antibodies to pancytokeratin, cytokeratin 7, cytokeratin 20, epithelial membrane antigen, vimentin, smooth muscle actin and CD-117. RESULTS: We observed three patients who presented with multiple metastases from large cell bronchial carcinoma to small intestine. Two of them had abdominal symptoms (sudden onset of abdominal pain, constipation and vomiting) and in one case the tumor was incidentally found during autopsy. Microscopically, all tumors showed a same histological pattern and consisted almost exclusively of strands and sheets of poorly cohesive, polymorphic giant cells with scanty, delicate stromas. Few smaller polygonal anaplastic cells dispersed between polymorphic giant cells, were also observed. immunohistochemistry showed positive staining of the tumor cells with cytokeratin and vimentin. Microscopically and immunohistochemically all metastases had a similar pattern to primary anaplastic carcinoma of the small intestine. CONCLUSION: In patients with small intestine tumors showing anaplastic features, especially with multiple tumors, metastases from large cell bronchial carcinoma should be first excluded, because it seems that they are more common than expected.- - - - - - - - - - ranking = 0.5keywords = antigen (Clic here for more details about this article) |
10/34. Postradiation angiosarcoma of the small intestine: a case report and review of literature.Postradiation angiosarcoma is typically a high-grade sarcoma that presents mainly in the skin and superficial tissues. Postradiation angiosarcoma arising in the small intestine is rare with only 11 cases documented in the English-language literature. Herein, we report a postradiation angiosarcoma of the small intestine 9 years after radiotherapy for uterine cervical adenocarcinoma. The patient presented with symptoms of intestinal obstruction. At exploratory laparotomy, tumor nodules involved the small bowel. Microscopically, the neoplasm was composed of spindled and epithelioid cells arranged in solid aggregates and focally forming vascular channels. The diagnosis of angiosarcoma was confirmed immunohistochemically by tumor cell expression of CD31, CD34, and factor viii-related antigen. The patient died 10 months after laparotomy. The diagnosis of PRA should be entertained for any poorly differentiated neoplasm arising in a previously irradiated site. The correct diagnosis of PRA depends upon histomorphologic identification of vascular differentiation, coupled with immunohistochemical expression of endothelial-related markers.- - - - - - - - - - ranking = 0.5keywords = antigen (Clic here for more details about this article) |
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