1/22. Multiple small intestinal stromal tumours in a patient with previously unrecognised neurofibromatosis type 1: immunohistochemical and ultrastructural evaluation.Neurofibromatosis type 1 could be associated with multiple gastrointestinal stromal tumours, although their presence is not considered among the major diagnostic criteria. We present here a case of a 50-year-old female complaining of abdominal pain, with about 100 small intestinal stromal tumours. This finding prompted us to suspect a neurofibromatosis which was clinically confirmed afterwards. light microscopy examination revealed a low-grade stromal tumour with skeinoid fibres. Mixed neural-interstitial cells of cajal origin or, alternatively, neural differentiation of interstitial cells of cajal are discussed on the basis of immunophenotype (CD117 , CD34 ) and ultrastructure. A 2-year follow-up did not indicate an aggressive course in the case of this neoplasm.- - - - - - - - - - ranking = 1keywords = gastrointestinal stromal tumour, gastrointestinal stromal, stromal tumour, stromal (Clic here for more details about this article) |
2/22. 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).- - - - - - - - - - ranking = 0.22365706529046keywords = gastrointestinal stromal, stromal (Clic here for more details about this article) |
3/22. Malignant gastric stromal tumor: unusual metastatic patterns.We present the case of a woman with metastatic malignant gastric stromal tumor occurring four years following a partial gastrectomy and distal esophagectomy, which presented as atypical breast mass with synchronous occult gastrointestinal bleeding. A discussion of metastatic pattern is presented, with emphasis placed on the need for continued surveillance after resectional surgery for gastrointestinal stromal tumor (GIST).- - - - - - - - - - ranking = 0.23668251246338keywords = gastrointestinal stromal, stromal (Clic here for more details about this article) |
4/22. Malignant fibrous histiocytoma of the abdominal cavity: report of a case.Malignant fibrous histiocytoma (MFH) is a soft-tissue sarcoma originating from fibroblast cells, characterized by a high rate of metastasis or recurrence. This tumor rarely develops in the gastrointestinal tract, with no more than 30 cases described in the literature. We report a case of MFH of the abdominal cavity in a 45-year-old woman who presented with epigastric pain, anorexia, and weight loss. A computed tomography (CT) scan of the abdomen revealed multiple solid tumors in the peritoneal cavity. We performed exploratory laparotomy and found at least 15 solid whitish tumors attached to the wall of the small intestine, as well as to the parietal peritoneum. There were three metastases in the liver. All of the tumors were excised, most of which were about 10 cm in diameter. Histopathological findings indicated a stromal tumor consisting of spindle cells, and immunohistochemical examination of the resected specimens established the definite diagnosis of a pleomorphic MFH. The patient had an uneventful postoperative course and was given adjuvant chemotherapy. She is currently well 2 years after her operation. We review the clinical picture of this tumor in the abdominal cavity, and discuss its diagnosis, pathogenesis, and treatment.- - - - - - - - - - ranking = 0.0026050894345848keywords = stromal (Clic here for more details about this article) |
5/22. Malignant tumors of the small intestine.SUMMARY: Malignant tumors of the small bowel are unusual and account for only 1% to 5% of all gastrointestinal tract malignancies. Thirteen cases of malignant tumors of the small bowel identified at the VAMC in puerto rico from January 1999 to September 2001 and a review of the literature are presented. The mean age of our cases was 67 (range: 45-78). Vague abdominal pain, nausea, vomiting, and melena were the most frequently reported symptoms. The average time from first symptoms to diagnosis was 3.2 months. A positive test for occult blood or hypochromic microcytic anemia was invariably present. hyperbilirubinemia and increased alkaline phosphatase were warning signs that allowed earlier diagnosis in patients with duodenal tumors. Fifty-four percent of the lesions were detected by endoscopic examination while 46% relied on radiographic studies. Eleven had carcinomas and two malignant gastrointestinal stromal tumors. Neither carcinoids nor lymphomas were identified. Our report of thirteen cases of malignant small bowel tumors is unusual and exhibits differences with the previously reported data. The cases were identified in a limited Hispanic population in a short period of time. Aggressive evaluation and a high suspicion of these malignancies should be entertained whenever subtle symptoms and unexplained gastrointestinal blood loss are assessed.- - - - - - - - - - ranking = 0.22365706529046keywords = gastrointestinal stromal, stromal (Clic here for more details about this article) |
6/22. 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 = 0.44731413058091keywords = gastrointestinal stromal, stromal (Clic here for more details about this article) |
7/22. Case of optic nerve involvement in metastasis of a gastrointestinal stromal tumor.BACKGROUND: The gastrointestinal stromal tumor (GIST) belongs to the leiomyosarcoma or leiomyoma group, and metastasis of leiomyosarcoma to the orbit is extremely rare. The most common sign of the metastasis of leiomyosarcoma to the orbit is proptosis; the primary lesion is usually found in skin or soft tissue. CASE: A 60-year-old man, who had a history of multiple metastases of GIST, suffered sudden unilateral blindness. OBSERVATIONS: His history and computed tomographic scans suggested that the lesion was a metastasis of GIST to the cavernous sinus around the root of the optic nerve. CONCLUSION: To the best of our knowledge, this is the first case of metastasis of GIST to the orbit.- - - - - - - - - - ranking = 1.1182853264523keywords = gastrointestinal stromal, stromal (Clic here for more details about this article) |
8/22. Multiple small intestinal stromal tumors associated with neurofibromatosis-1.gastrointestinal stromal tumors (GISTs) are rarely noted in association with neurofibromatosis-1 (NF-1, von Recklinghausen disease) as an individual gastrointestinal manifestation. We report here a case of multiple GISTs with an abundant skeinoid fiber in the jejunum of a 43-year-old woman diagnosed as NF-1. Histologically, the tumors were composed of uniform spindle-shaped cells with a fascicular pattern, almost indistinguishable from the histology characteristic of usual GISTs. However, multiple synchronous tumor occurrence, abundant skeinoid fiber, and presence of microscopic miniatures of stromal tumors are additional characteristic features of this case.- - - - - - - - - - ranking = 0.015630536607509keywords = stromal (Clic here for more details about this article) |
9/22. Spindle cell sarcoma: a rare cause of a large abdominal mass.A 73-year-old man presented with a huge intra-abdominal mass. Initially a gastrointestinal stromal tumour (GIST) was diagnosed, but his tumour was subsequently classified as a spindle cell sarcoma. Difficulties in the classification of rare intra-abdominal tumours are discussed.- - - - - - - - - - ranking = 0.6484483148837keywords = gastrointestinal stromal tumour, gastrointestinal stromal, stromal tumour, stromal (Clic here for more details about this article) |
10/22. gastrointestinal stromal tumors metastatic to the ovary: a report of five cases.Five cases of gastrointestinal stromal tumor metastatic to the ovary are reported. The average patient age was 59 years (range, 44-81 years). The primary tumor was in the small bowel or its mesentery (4 cases) or stomach (1 case). The primary and metastatic tumors were discovered synchronously in 3 cases. In the other 2 cases, the ovarian tumors were discovered 18 months before a gastric tumor was identified and 27 years after a small bowel tumor had been resected. The ovarian tumors (three of which were bilateral) were usually solid, tan, and lobulated. Microscopically, three tumors had a pure spindle cell morphology, and two both spindle and epithelioid cell components. The diagnosis in all 5 cases was confirmed with positive c-kit (CD117) and negative desmin immunostaining. Variably positive immunoreactivity for either or both h-caldesmon and smooth muscle actin was seen in all 5 cases, and 3 cases were CD34-positive. Four patients died between 1 and 6.5 years (mean, 2.8 years) from the time of ovarian tumor diagnosis. The main differential diagnostic consideration was leiomyosarcoma; the most important features to help exclude this diagnosis were an absence of tumor in the uterus, low histologic grade, and a desmin-negative, c-kit-positive immunophenotype. Other differential considerations, including endometrial stromal sarcoma and fibrosarcoma, are discussed. Most of the ovarian tumors in this series were initially diagnosed as tumors of other types, a misdiagnosis with significant therapeutic and prognostic implications because of the specific therapy now available for gastrointestinal stromal tumors.- - - - - - - - - - ranking = 0.46033957775383keywords = gastrointestinal stromal, stromal (Clic here for more details about this article) |
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