Cases reported "Duodenal Neoplasms"

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1/12. Prostate cancer metastasizing to the small bowel.

    Although prostate cancer is one of the most commonly encountered malignancies in clinical practice, it is very unusual for prostate cancer to metastasize to the small bowel. Our search of the literature found no such cases published from 1966 to the present. We report the case of a 69-year-old man who presented for evaluation of anasarca and anorexia. He had a history of prostate cancer diagnosed 9 years before and had undergone a radical prostatectomy with subsequent radiotherapy for positive tumor margins. He developed anasarca 2 years before presentation to us. His serum albumin ranged between 1.5 and 2.5 g/dL. Upper endoscopy was performed for possible protein-losing enteropathy and the appearance of gastric and duodenal mucosa was found to be normal. Random small bowel biopsies revealed submucosal infiltrating adenocarcinoma with positive prostate-specific antigen stains consistent with the diagnosis of prostate cancer metastatic to the small bowel. This is a rare presentation of metastatic prostate cancer. Even though prostate cancer is the most commonly diagnosed cancer in American men, antemortem diagnosis of small bowel metastasis has not been reported. In patients with unexplained anasarca, especially with a history of malignancy, an upper endoscopy with small bowel biopsy may be useful in establishing the diagnosis.
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2/12. Concurrent occurrence of gastric adenocarcinoma and duodenal neuroendocrine cell carcinoma: a composite tumour or collision tumours ?

    BACKGROUND: Neuroendocrine cell (NEC) carcinoma is occasionally accompanied by adenocarcinoma but the relationship between these two morphologically distinct tumours is unclear. Two hypotheses have arisen regarding the mechanism for the association of adenocarcinoma and NEC carcinoma. One is that both are derived from a common multipotential epithelial stem cell. The second hypothesis is that adenocarcinoma and NEC carcinoma arise from a multipotential epithelial stem cell and a primitive NEC, respectively. AIMS: To elucidate the relationship between the two histologically distinct tumours, adenocarcinoma of the stomach and NEC carcinoma of the duodenum. PATIENT/methods: We present a case in which the tumour extended across the pyloric ring, the gastric portion of which revealed adenocarcinoma while the duodenal portion showed argyrophil NEC carcinoma. The two histologically distinct lesions of the tumour were examined by immunohistochemistry and genetic analysis of p53. RESULTS: The gastric region was negative for chromogranin a staining but positive for carcinoembryonic antigen (CEA) staining. In contrast, the duodenal region was positive for chromogranin a but negative for CEA. All tumour regions showed a point mutation in p53 gene at exon 7 (GGC (glycine)-->GTC (valine) at codon 245). The distal portion of the duodenal tumour showed an additional point mutation in p53 gene at exon 5 (GCC (alanine)-->GTC (valine) at codon 129). CONCLUSIONS: The two histologically distinct tumours, adenocarcinoma of the stomach and NEC carcinoma of the duodenum, appear to be derived from a common epithelial cell.
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3/12. Hepatoid adenocarcinoma of the papilla of Vater.

    An adenocarcinoma of the papilla of Vater showing hepatoid differentiation similar to that described for hepatoid adenocarcinoma of the stomach is reported. The tumor was a poorly differentiated adenocarcinoma with clear cells containing occasional hyaline droplets and exhibiting bile secretion. Alpha-fetoprotein (AFP), alpha 1-antitrypsin (alpha 1-AT) and carcino-embryonic antigen (CEA) were demonstrated by immunoperoxidase staining. This appears to be the first report of a hepatoid adenocarcinoma at this location.
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4/12. Interdigitating dendritic cell sarcoma of the duodenum with rapidly fatal course: a case report and review of the literature.

    Interdigitating dendritic cell sarcoma is an extremely rare malignancy derived from antigen-presenting cells. dendritic cells constitute a heterogeneous group of cells, which includes langerhans cells, dermal dendrocytes, follicular dendritic cells, and interdigitating dendritic cells present in lymphoid and nonlymphoid organs. We report the case of a 36-year-old woman who presented with epigastric pain, projectile vomiting, and significant weight loss. Upper gastrointestinal endoscopy showed a duodenal lesion; a biopsy of the lesion was taken and was diagnosed as sarcoma. She underwent a Whipple procedure. A final diagnosis of interdigitating dendritic cell sarcoma was made, with liver and peripancreatic lymph node involvement. The patient deteriorated rapidly and died 4 months later. Although interdigitating dendritic cell sarcoma of the duodenum is extremely rare, we think it should be included in the differential diagnosis of unusual spindle cell tumors with a rich lymphocytic infiltrate.
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5/12. carcinoid tumor arising in a duplication cyst of the duodenum.

    Reported herein is a case of carcinoid tumor arising in a duplication cyst of the duodenum in a 34-year-old woman. ultrasonography at a health check-up revealed a 10 cm cystic mass located in the retroperitoneum adjacent to the third portion of the duodenum. Macroscopically, it had a smooth surface without communication with other organs and was filled with brownish necrotic fluid. More than half of the inner wall was occupied by a white, irregular, and solid nodule, which protruded into the lumen. The nodule was diagnosed as a carcinoid tumor invasive to the deep muscular layer. The non-neoplastic cyst wall had bowel structures including mucosa, submucosa and double layers of smooth muscle, indicating that the lesion was a duodenal duplication cyst. Most of the mucosa was eroded by marked inflammation. The remaining mucosa consisted of various types of epithelium, the major type having the nature of primitive gastric mucosa. Of interest was the presence of hamartomatous components in the wall. Furthermore, the non-neoplastic mucosal lining cells around the carcinoid tumor expressed neuroendocrine antigens and had minimal proliferative activity, suggesting that part of the cyst wall provided a microenvironment for accelerated differentiation of epithelial cells to an endocrine phenotype and transformation to neoplasia.
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6/12. Duodenal gangliocytic paraganglioma. Report of a case and review of the literature.

    A case of gangliocytic paraganglioma (GP) of the ampulla of vater is reported and the literature reviewed, with special attention to immunohistochemical studies. The present case, which occurred in a 56-year-old woman, shows the typical histological admixture of epithelioid, ganglion and spindle cells. immunohistochemistry reveals strong reactivity for synaptophysin, Leu-7, somatostatin, S-100 protein and vimentin. A few ganglion cells are reactive for neurofilaments. chromogranin a, myelin basic protein, desmin and cytokeratin are absent. Immunohistochemical data from literature regarding the cytoskeletal composition of GPs are not unequivocal: cytokeratin and neurofilament positivity is reported by some authors and denied by others. More uniformity is reported concerning the peptides produced by GPs: somatostatin and pancreatic polypeptide are the most frequently found antigens, followed by serotonin. General neuroendocrine markers like neuron specific enolase and protein gene product 9.5 are always positive, whereas chromogranins are rarely found. S-100 protein is always positive in the spindle cell component. Our data are in keeping with those previously reported and add the diffuse positivity for the Leu-7 antigen and the positivity of ganglion cells for synaptophysin. The nature of the tumour is still a matter of debate and it is difficult to agree with either of the proposed hypotheses--hamartoma/choristoma versus true neoplasm. However the recent reports of the occasional malignant evolution of GPs may support their true neoplastic nature.
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7/12. Primary duodenal small-cell neuroendocrine carcinoma with production of vasoactive intestinal polypeptide.

    A primary duodenal small-cell neuroendocrine carcinoma was found in an elderly man who presented with upper abdominal pain. Although metastatic small-cell carcinoma was documented by liver biopsy, the primary lesion was not identified until postmortem examination. The latter tumor, which ulcerated the duodenal mucosa, was composed of small ovoid cells with sparse cytoplasm and granular chromatin. Electron microscopy revealed cytoplasmic dense-core granules. Immunocytochemical study demonstrated the presence of neuron-specific enolase, Leu 7 antigen, chromogranin, epithelial membrane antigen, and vasoactive intestinal polypeptide within tumor cells. However, there was no evidence of a clinical endocrinopathy. This case emphasizes the need to include the duodenum as a possible primary site when metastatic small-cell neuroendocrine carcinoma is seen in the absence of apparent pulmonary disease.
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8/12. Ampullary carcinoma associated with multiple duodenal villous adenomas.

    A 39-year old Asian with multiple duodenal villous adenomas and an adenocarcinoma arising from one of these tumors is described. The rarity of villous adenomas at this site is stressed and their tendency to undergo malignant change reviewed. Immunoperoxidase studies using carcinoembryonic antigen (CEA) antiserum were positive in high dilutions (1 in 8,000) in the carcinoma and the largest adenoma. This stain would appear to show functional malignancy before histological evidence of malignancy is found.
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9/12. Epithelioid angiosarcoma of the intestinal tract with endothelin-1-like immunoreactivity.

    Two rare cases of intestinal epithelioid angiosarcoma arising in the sigmoid colon and small intestine are reported. The small intestinal tumours were located in the duodenum and upper jejunum. All tumours showed reddish-black protuberant masses with comparatively clear margins. histology reveals solid and epithelioid growth of large polygonal cells, mimicking undifferentiated carcinoma. Vascular differentiation such as lamina containing red cells and intracytoplasmic vacuolization is noticed. Intense immunoreactivity to ulex europaeus agglutinin I and JC70 (CD31), and sporadic positive reaction to factor viii related antigen were detected in one case but not in the other. However, both tumours have cells which show intense endothelin-1 immunoreactivity. Positive immunostaining occurred with low molecular weight keratins suggesting epithelial differentiation and suggested epithelioid angiosarcoma as a diagnosis, a specific entity within angiosarcoma. Because of their histological features, epithelioid angiosarcomas may be confused with undifferentiated carcinomas. endothelin-1-like immunoreactivity seems to be a good marker for this type of angiosarcoma, even when the tumour cells lack usual marker substances for endothelial cells.
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10/12. Tripartite differentiation in a carcinoma of the duodenum.

    BACKGROUND. Carcinomas containing three distinctly different cell lines have been encountered in the colon and rectum, but a tripartite malignancy in the small intestine has not been reported previously. methods. A duodenal carcinoma was studied by light and electron microscopic examination and immunohistochemistry. RESULTS. The duodenal carcinoma was found to have tripartite glandular, squamous, and neuroendocrine differentiation. Histologically, an adenocarcinoma, which originated in a villous adenoma, was continuous with squamous cell carcinoma and small cell carcinoma components. Tumor cells of the squamous cell carcinoma component had conspicuous intercellular bridges but did not form keratin pearls. Immunohistochemical analysis showed strong expression of carcinoembryonic antigen (CEA) by the adenocarcinomatous component. The squamous cell carcinoma component demonstrated focal weak CEA and neuron specific enolase (NSE) reactivity. Ultrastructurally, tumor cells of this component had frequent desmosomes and free tonofilaments. The small cell carcinoma had clusters of dense core granules in tumor cell cytoplasmic processes, which are indicative of neuroendocrine differentiation. This neuroendocrine component was immunoreactive for somatostatin and NSE. CONCLUSIONS. This case of tripartite duodenal carcinoma supports the theory of an origin from an intestinal pluripotential stem cell capable of differentiating into multiple cell types.
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