Cases reported "Intestinal Neoplasms"

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1/13. A case of carcinoid.

    A patient is described with a malignant carcinoid tumour of the ileum with nodal secondaries causing mesenteric vessel occlusion and ileal infarction.
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2/13. 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.
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3/13. Intraabdominal lymphangiomyoma in an infant with protein-losing enteropathy and hemihypertrophy.

    lymphangiomyoma is an extremely rare tumor occurring exclusively in women of reproductive age. The tumor is characterized by proliferation of immature smooth muscle along the lymphatic vessels of the abdomen, thorax and lung. Although lymphangiomyoma has been reported in a young girl and a girl infant, none has been reported in boys. We report herein a case of lymphangiomyoma in a two-year-old boy. The unusual presentation in this patient was that the tumor arose from the small bowel mesentery without any evidence of lung involvement. The tumor was extirpated and lymphangiomyomatosis was confirmed pathologically.
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4/13. Small-intestinal sarcomatoid carcinoma with superior vena cava syndrome.

    A 56-year-old man was hospitalized because of swelling of the right upper extremity and anemia. A diagnosis of superior vena cava (SVC) syndrome caused by lymphogenous metastasis was made after chest computed tomography (CT) scan and biopsy of cervical lymph nodes were carried out. Standard examinations, such as abdominal CT scan and endoscopies of the upper and lower gastrointestinal tract, failed to find the primary lesion. However, selective angiography of the superior mesenteric artery (SMA) showed a clear stain of bleeding vessels in the small intestine. laparotomy was performed, and immunohistochemical findings revealed sarcomatoid carcinoma in the small intestine (a rarely seen neoplasm). This aggressive carcinoma, which showed negative reactivity with CD34, CD117 (c-kit), and S-100 was clearly distinguished from other mesenchymal tumors, such as malignant gastrointestinal stromal tumor (GIST) and malignant fibrous histiocytoma (MFH).
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5/13. 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.
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6/13. Reactive angioendotheliomatosis of the intestine.

    We present a case of reactive angioendotheliomatosis (RAE) of the colon, featuring intravascular proliferation of endothelial cells with histologic resemblance to glomeruloid hemangioma. A 19-year-old Japanese male with an anal fistula was diagnosed endoscopically with Crohn's disease. Six months later, he was hospitalized for fever and abdominal pain. Emergency resection of ileocecum and splenic flexure of the colon was undertaken to control massive intestinal hemorrhage, and in all parts of the resected colon, foci of many small vessels with intravascular proliferation of endothelial cells were noted throughout the layers. Moreover, solid proliferation of endothelial cells was seen in the submucosa at the base of open ulcers. Two small granulomas, compatible with Crohn's disease, were also evident in the muscle layer of the terminal ileum. No other hemangiomas or hemangioma-like structures were observed with CT scans, and the vascular lesions were histologically diagnosed as RAE. The pathogenesis of this disorder is unknown, and most cases occur in skin with systemic disease. The present case might thus be a first case of RAE of the intestine without cutaneous involvement. Whether there is a relation with coexistent enteritis suggestive of Crohn's disease needs to be clarified.
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7/13. Combined leiomyomatosis of the small intestine and colon.

    We report a case of leiomyomatosis that involved the small intestine and colon. On gross examination, the bowel wall was irregularly thickened at various levels. Histologically, the lesions were characterized by multifocal, diffuse tumor masses that were present in the muscular layers. These masses consisted of proliferating smooth-muscle cells that often surrounded prominent blood vessels. Cytologic atypia was not observed, and the mitotic count was low, illustrating the benign nature of the tumors. Findings from immunohistochemical analysis of the lesions were essentially negative; vimentin, cytokeratin, actin, desmin, and S100 stains showed no reactivity in the tumor cells, despite positive internal controls. By reviewing the literature, we found several reports on related lesions that occurred in the gastrointestinal tract; diffuse leiomyomatosis of the esophagus and the colon have been reported. We did not find any report on the small intestinal variant of the disease; therefore, it seemed useful to term this new localization as leiomyomatosis of the small intestine and colon.
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8/13. Laparoscopic-assisted small bowel resection of a leiomyoma causing recurrent obscure gastrointestinal bleeding.

    A 62-year-old man experienced recurrent painless episodes of melenas and undiagnosed chronic anemia for 4 years. Despite extensive radiologic and endoscopic work-up, the origin of the bleeding could not be identified. At his last admission, visceral angiography revealed an area of hypervascularity at the initial portion of the jejunum, containing irregular, corkscrew vessels coming from the first jejunal branch of the superior mesenteric artery. Under general endotracheal anesthesia, a 10-mm trocar for the 30 degree laparoscope was inserted subumbilically using the open Hasson technique. Two 5-mm trocars were also used suprapubically and at the left iliac fossa. A 2.5 cm diameter, smooth, hypervascular tumor was easily visualized on the jejunal wall 10 cm from the ligament of Treitz. The small bowel segment was laparoscopically mobilized and brought through the subumbilical trocar site, which was extended 1 cm. A limited small bowel resection and a stapled anastomosis were easily performed extracorporeally. Histopathologic examination diagnosed a benign gastrointestinal tumor (gastrointestinal stromal tumor, leiomyoma). The patient was discharged on postoperative day 2. Laparoscopic identification and mobilization allows a loop of small bowel to be exteriorized through a small incision; the anastomosis can be safely performed extracorporeally. The advantages of rapid postoperative recovery and reduced pain are evident.
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9/13. Angiotropic large cell lymphoma (angioendotheliomatosis) presenting with protein-losing enteropathy.

    A 73 year old female presented with progressive hypoproteinaemic oedema which was resistant to treatment. The cause was not established until post-mortem, when it was found that she had angiotropic large cell lymphoma involving the vessels of the large intestine with mucosal ulceration. To our knowledge, this is the first report of angiotropic large cell lymphoma, which usually presents with neurological and skin involvement, presenting with protein-losing enteropathy.
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10/13. Intestinal ischaemia associated with ileal carcinoid tumours.

    A case of intestinal infarction due to multiple ileal carcinoid tumours is presented in which abdominal pain due to intestinal ischaemia was the first manifestation of the underlying tumour. It is postulated that intestinal ischaemia may be of more importance in the production of abdominal pain by carcinoid tumours than has been generally accepted, and that it is the result of functional and structural changes in the around the mesenteric blood vessels, caused by substances secreted by the carcinoid tumour.
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