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11/49. Reactive nodular fibrous pseudotumor of the gastrointestinal tract and mesentery: a clinicopathologic study of five cases.

    Although the majority of mesenchymal lesions of the gastrointestinal tract are neoplastic in nature, nonneoplastic reactive processes may involve the gastrointestinal tract and mesentery, causing diagnostic confusion with more aggressive neoplasms, such as fibromatosis or gastrointestinal stromal tumors. In this study, we report a series of fibroinflammatory lesions of the gastrointestinal tract that we think represent a relatively cohesive group of tumors and describe the clinical and pathologic features of this entity, which we have termed "reactive nodular fibrous pseudotumor." The tumors affected five patients (four male and one female patient) who ranged in age from 48 to 71 years (mean 56 years). Two patients presented with acute abdominal pain without a significant past medical history, two had incidental lesions discovered during evaluation for other medical conditions, and one was found to have an abdominal mass. Three patients had a history of abdominal surgery. The tumors were multiple in three patients and solitary in two patients. In four cases, at least one of the tumors involved the small intestine or colon, and the lesion was confined to the peripancreatic soft tissue in one case. The tumors were firm, tan-white, ranged in size from 4.3 to 6.5 cm in greatest dimension, and were grossly well circumscribed. All of the lesions were of low to moderate cellularity and composed of stellate or spindled fibroblasts arranged haphazardly or in intersecting fascicles. Three cases had microscopically infiltrative borders. The stroma was rich in collagen, which was wire-like, keloidal, or hyalinized. Intralesional mononuclear cells were sparse but were more numerous peripherally and frequently arranged in lymphoid aggregates. Immunohistochemical stains demonstrated that all of the tumors stained for vimentin, 80% stained for CD117 or muscle specific actin, 60% stained for smooth muscle actin or desmin, and none of the tumors stained for CD34, S-100 protein, or anaplastic lymphoma kinase-1. Follow-up information was available in all cases: four patients had no residual disease following surgical resection (mean follow-up 16.3 months) and one patient who had an incomplete surgical resection had stable disease at 26 months. In summary, we report a series of distinct intraabdominal fibroinflammatory pseudotumors that we have collectively termed "reactive nodular fibrous pseudotumors." These lesions are uncommon and may infiltrate the bowel wall, thereby mimicking primary bowel neoplasms or intraabdominal fibromatosis. Recognition of these nonneoplastic lesions is important, as they pursue a benign clinical course, but may be confused with other mesenchymal neoplasms that require more aggressive treatment.
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12/49. A rare case of multiple lymphomatous polyposis with widespread involvement of the gastrointestinal tract.

    Multiple lymphomatous polyposis (MLP) is an uncommon type of primary non-Hodgkin gastrointestinal (GI) B-cell lymphoma characterized by the presence of multiple polyps along the GI tract. Malignant cells of MLP have mantle cell characteristics and thus are considered to be the counterpart of the mantle cell lymphoma (MCL) in the GI tract. Since 1961, no more than 70 well-documented cases have been published. We report the case of 53-year-old man diagnosed as having MLP. The patient presented with diffuse abdominal pain, chronic lower GI bleeding, peripheral lymphadenopathy, and weight loss. The lymphomatous polyps extended from the esophagus to the rectum, with bone marrow infiltration. Immunohistologic findings were characteristic of MCL. The patient was treated with a combined cyclophosphamide, vincristine, and prednisone chemotherapy regimen, resulting in a partial response.
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13/49. Treatment with STI571, a tyrosine kinase inhibitor, for gastrointestinal stromal tumor with peritoneal dissemination and multiple liver metastases.

    BACKGROUND: gastrointestinal stromal tumors (GISTs) are usually refractory to standard chemotherapeutic agents. We successfully treated a patient with a tyrosine kinase inhibitor (STI571) for GIST with peritoneal dissemination and liver metastases. methods: In a 32-year-old man presenting with abdominal pain from diffuse peritonitis, a GIST and associated perforated small intestine were resected. Multiple liver metastases were present. After therapies with microwave coagulation, ethanol injection, and local and systemic antineoplastic drugs (fluorouracil, cisplatin, tegafur-uracil, and tegafur) failed, investigational treatment with a tyrosine kinase inhibitor was initiated (STI571, 300 mg, p.o. daily). RESULTS: anorexia and abdominal fullness resolved within a few days. At 24 days after initiation, positron emission tomography showed a remarkable decrease in the abdominal uptake of [18F] fluorodeoxyglucose. Adverse effects of STI571, including mild alopecia and anemia, were minimal. CONCLUSIONS: The tyrosine kinase inhibitor STI571 may be effective against GISTs.
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14/49. Exophytic pedunculated gastrointestinal stromal tumor with remarkable cystic change.

    A 59-year-old man with bloody stools, and previously diagnosed with sigmoid colon carcinoma, visited our hospital. Preoperative abdominal ultrasonography (US) showed another tumor, with an uneven irregular surface, measuring about 9 x 5 cm, below the left hypochondrium. The tumor consisted of several cysts. Abdominal computed tomography (CT) showed a multicystic tumor attached to the stomach, and its septum and marginal region were intensely stained on contrast imaging. On magnetic resonance imaging (MRI), low and markedly high signals were revealed in the tumor on T1-weighted and T2-weighted sequences, respectively. Contrast imaging of the upper digestive tract showed extramural compression of the greater curvature of the antral stomach by the tumor. The tumor was partially imaged by endoscopic ultrasonography (EUS), but continuity to the stomach was not confirmed. On abdominal angiography, the tumor was slightly stained via the gastroepiploic arteries. Surgical treatment was performed to excise both the gastric tumor and the sigmoid colon carcinoma. The gastric tumor was removed with gastric wall tissue where the tumor was attached to a 2-cm pedicle. It was multicystic, contained watery fluid, and had a smooth outer surface. Histologically, the tumor consisted of multiple irregular cysts without epithelial lining, and solid epitheloid cell nests in between. The tumor cells had clear or eosinophilic cytoplasm and round nuclei. No mitotic figures were seen. The tumor cells in the pedicle were connected with the muscularis propriae of the stomach. immunohistochemistry showed c-kit-positive, CD34-positive smooth muscle actin (SMA)-negative, and S-100-negative staining of tumor cells. The final diagnosis was gastrointestinal stromal tumor (GIST).
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keywords = upper
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15/49. Needle catheter jejunostomy complicated by pneumatosis intestinalis: a case report.

    Needle catheter jejunostomy is a technique to allow enteral feeding after upper abdominal surgery. An unusual postoperative complication is pneumatosis intestinalis which can be life-threatening. A case of a 76-year-old man with pneumatosis intestinalis due to needle catheter jejunostomy, in whom diagnosis was made by CT-scan, is described. Needle catheter jejunostomy complicated by pneumatosis intestinalis needs attention and careful treatment. Removal of the catheter seems necessary, although controversy remains.
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keywords = upper
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16/49. Malignant gastrointestinal stromal tumor originating in the lesser omentum, complicated by rapidly progressive glomerulonephritis and gastric carcinoma.

    A 69-year-old man was admitted with a large elastic mass in the upper abdomen. Computed tomography revealed a massive tumor in contact with the liver and gastrointestinal endoscopy revealed a gastric adenocarcinoma. He developed acute renal failure with massive proteinuria and died with a marked enlargement of the tumor. autopsy revealed a tumor located in the lesser omentum. The tumor was considered to be a Gastrointestinal Stromal Tumor (GIST) because it was positive for c-kit. In addition, crescent formations and immune complexes in glomeruli were observed. We report the first case of GIST complicated by rapidly progressive glomerulonephritis and gastric carcinoma.
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ranking = 0.12211772169217
keywords = upper
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17/49. Acute onset of non-Hodgkin's lymphoma with serious gastrointestinal haemorrhage: diagnosis and surgical treatment (case report).

    The authors report a rare case of acute onset of gastrointestinal non-Hodgkin's lymphoma with acute gastrointestinal haemorrhage. The patient, a man aged 49 years, was admitted to the surgical department for the evaluation of an increased anemia and weakness. physical examination disclosed mild epigastric tenderness in response to palpation but no palpable mass. Few hours after hospital admission, he underwent a serious gastrointestinal haemorrhage with shock. laparotomy revealed that the malignant lesions were in the upper and in the middle of the stomach and multiple white lesions were found in the small bowel. A standard radical gastrectomy with D2 lymphadenectomy and bowel resection (about 120 cm) was performed; bowel canalization was restored by esophago-jejunal end-to-side anastomosis Roux-en-Y. The main problems of surgical treatment are discussed.
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ranking = 0.12211772169217
keywords = upper
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18/49. Gastrointestinal metastases from malignant melanoma: report of a case.

    Malignant melanoma metastases in the gastrointestinal tract (GIT) are found in more than 60% of autopsies on patients who have died with disseminated melanoma; however, the rate of GIT metastases detected clinically averages only 2%. This discrepancy seems to be attributed to the nonspecific symptoms and signs of GIT involvement, which include weakness, fatigue, bleeding, anemia, and abdominal pain. Sometimes a diagnosis is only made when bowel obstruction occurs. We report a case of long-term survival after surgery for multiple melanoma metastases in the gastrointestinal tract and review the relevant literature. Both our case report and the literature review demonstrate the benefits of surgery for patients with melanoma metastases in the GIT. We also stress the need for meticulous follow-up, detailed history-taking, and rapid evaluation of any vague and unclear abdominal signs and symptoms for patients with melanoma.
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19/49. Emergency pylorus-preserving pancreatoduodenectomy followed by second-stage pancreatojejunostomy for a gastrointestinal stromal tumor of the duodenum with an intratumoral gas figure: report of a case.

    We report a case of a duodenal gastrointestinal stromal tumor (GIST) necessitating urgent surgery because of a gas figure on computed tomography (CT). A 46-year-old woman, complaining chiefly of upper abdominal pain and tarry stools, consulted a local doctor. A gastrointestinal fiberscopy revealed an ulcer in the second part of the duodenum, and the patient was admitted to our hospital where a dynamic CT scan showed a hypervascular solid tumor in the pancreatic head. A repeat CT scan done 4 days later showed a gas figure in the tumor, necessitating an emergency pylorus-preserving pancreatoduodenectomy (PpPD). First, we performed a tube pancreatostomy for complete external drainage of the pancreatic juice, and planned a second-stage pancreatojejunostomy for the near future. Histopathologically, the tumor was diagnosed as a GIST originating in the duodenum. The patient was discharged on postoperative day 23 after an uneventful postoperative recovery. Her local doctor completed the second-stage pancreatojejunostomy.
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ranking = 1.1221177216922
keywords = abdominal pain, upper
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20/49. Neurofibromatosis with gastrointestinal stromal tumors: insights into the association.

    The frequent association of stromal tumors with neurofobromatosis raises high suspicion of a possible correlation between the two entities. The aim of this study was to analyze clinicopathologic features of patients with concomitant neurofibromatosis and gastrointestinal stromal tumors and to discuss the molecular basis for their possible pathogenesis. Detailed information about clinical presentation, histology, immunostains, polymerase chain reaction amplification, and sequencing in three of our own cases was obtained. Stromal tumors presented with abdominal pain in one case and hemorrhage in another. One patient underwent surgery for malignant transformation of neurofibroma and stromal tumors were found incidentally. Stromal tumors were consistently positive for CD117, while the malignant peripheral sheath tumor was not. mutation in the KIT juxtamembrane domain was found in one case. In this respect, some stromal tumors lack demonstrable KIT mutations but KIT remains activated. We reasoned that other mechanisms, like the Ras pathway involved in neurofibromatosis type 1, might play a role in KIT activation.
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