1/20. Duodenal periampullary gangliocytic paraganglioma: report of two cases with immunohistochemical and ultrastructural study.We report two cases of Gangliocytic paraganglioma (GP) of the ampulla of vater occurring in a 63-year-old and a 34-year-old individual. The patients were both admitted for a long history of intermittent gastrointestinal bleeding and abdominal discomfort, with no other symptoms. At endoscopy, the GP appeared as a polypoid, ulcerated mass in the ampullar region, measuring 2.5x1.8 and 2 cm, respectively. Microscopically, the tumors showed similar features and were composed of epithelial cells (more than 50%), spindle cells, and ganglion-like cells. The epithelial cells showed clear cytoplasm and formed nests (zellballen or paraganglioma-like groups), and less frequently, cords (carcinoid-like), extending to mucosa and submucosa. Ganglion cells were sparse, constantly associated with the spindle cells. Both epithelial and ganglion cells were synaptophysin, chromogranin a, and anti-neurofilament immunoreactive. The spindle cells were all S-100 positive. Ultrastructural studies revealed dark and light cells, rare elongated cellular processes, secretory granules, and fine fibrils resembling neurofilaments. The histogenesis of GP is still a matter of debate, however its neoplastic nature is supported by the occasionally reported malignant evolution.- - - - - - - - - - ranking = 1keywords = spindle cell, spindle (Clic here for more details about this article) |
2/20. 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 = 0.33333333333333keywords = spindle cell, spindle (Clic here for more details about this article) |
3/20. Small intestinal angiosarcoma leading to perforation and acute abdomen: a case report and review of the literature.A 76-year-old man presented to the emergency room with abdominal pain and fatigue. The patient had a history of gastrointestinal bleeding. An abdominal computed tomographic scan showed collections of free air in the abdomen with obstruction of the distal small intestine and multiple masses in the liver. laparotomy revealed a widespread malignant neoplasm in the abdomen, with multiple tumor nodules in the ileal wall, mesentery, and liver. The ileal wall was perforated within the area of one of the tumors. Pathologic examination of the lesion showed a neoplasm composed of solid sheets of spindle and undifferentiated cells, forming interlacing delicate vascular channels with atypical endothelial cells. The tumor cells were positive for the endothelial marker CD31, whereas reactivity for a broad panel of epithelial and other endothelial markers was negative. This case demonstrates that although angiosarcomas of the gastrointestinal tract are rare, they should be considered in cases of intestinal perforation or severe bleeding, especially in the elderly. A broad panel of immunochemical markers might be necessary to establish the pathologic diagnosis.- - - - - - - - - - ranking = 0.084583994564862keywords = spindle (Clic here for more details about this article) |
4/20. 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 = 0.33333333333333keywords = spindle cell, spindle (Clic here for more details about this article) |
5/20. 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 = 0.084583994564862keywords = spindle (Clic here for more details about this article) |
6/20. 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 = 0.33333333333333keywords = spindle cell, spindle (Clic here for more details about this article) |
7/20. 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 = 0.4179173278982keywords = spindle cell, spindle (Clic here for more details about this article) |
8/20. Localized intra-abdominal fibromatosis of the small bowel mimicking a gastrointestinal stromal tumor: a case report.Intra-abdominal fibromatosis (IAF) is a benign mesenchymal lesion that can occur throughout the gastrointestinal tract. Although rare, it is the most common primary tumor of the mesentery and can develop at any age. We describe a rare case of primary IAF involving the mesentery and small bowel which clinically, macroscopically and histologically mimicked malignant gastrointestinal stromal tumor (GIST). This report highlights the fact that benign IAF can be misdiagnosed as a malignant GIST localized in the mesentery or arising from the intestinal wall. Their diagnostic discrimination is essential because of their very different biological behaviors and the fact that the introduction of effective therapies involving tyrosine kinase inhibitor STI571 (imatinib mesylate) has greatly changed the clinical approach to intra-abdominal stromal spindle cell tumors.- - - - - - - - - - ranking = 0.33333333333333keywords = spindle cell, spindle (Clic here for more details about this article) |
9/20. 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 = 0.084583994564862keywords = spindle (Clic here for more details about this article) |
10/20. Pleomorphic carcinoma of the small bowel. The limitations of immunohistochemical specificity.We report 3 cases of poorly differentiated tumors of the small bowel with histological, immunohistochemical, and ultrastructural studies. The patients were male, aged 45, 57, and 63. In all 3 cases, histological features of spindle cell, epithelioid cell and giant cell areas favoured a diagnosis of carcinoma, although a malignant stromal tumor could not be firmly excluded. immunohistochemistry demonstrated in the 3 cases a strong expression of both "epithelial" (cytokeratin) and "stromal" (vimentin) markers; one tumor expressed the epithelial membrane antigen, and another one desmin. Electron microscopy showed no specific features in one case. The case positive for desmin demonstrated intracytoplasmic lumina, allowing the diagnosis of carcinoma. In spite of a non-specific immunohistochemical pattern, we finally considered these 3 tumors as of epithelial origin, corresponding to the rare and recently described pleomorphic carcinoma of the small bowel. This report emphasizes the difficult diagnosis of some poorly differentiated tumors, particularly in the gastro-intestinal tract. Such problems had until recently been resolved by ultrastructural and mostly by immunohistochemical studies. However, an increasing number of reports, together with our 3 cases, show unexpected reactivity of tumors with theoretically specific immunoreactions, such as those directed against intermediate filaments. Coexpression of intermediate filaments could be due to cross reactivity of molecules bearing common epitopes, or to the presence of different filaments in the same cell type; recent immunoblotting studies favour this latter hypothesis.- - - - - - - - - - ranking = 0.33333333333333keywords = spindle cell, spindle (Clic here for more details about this article) |
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