1/8. Gastrointestinal autonomic nerve tumours: a case report with ultrastructural and immunohistochemical studies.A case of gastrointestinal autonomic nerve tumour with light microscopic, immunohistochemical and ultrastructural examination is reported. The tumour was composed of spindle cells or large cells with clear cytoplasm and showed intense staining for vimentin and focal staining for neuron-specific enolase, chromogranin, synaptophysin, gastrin, P substance and S-100 protein. Ultrastructural examination showed long processes with dense core granules and the absence of features characteristic of other gastrointestinal stromal tumours. In addition we noted small traces of basal lamina and the absence of synaptic vesicles. It seems that the biological behaviour of gastrointestinal autonomic nerve tumours is aggressive but there are too few reports on which to conclude anything about their prognosis. Our findings suggest that tumour has a neuroectodermal differentiation.- - - - - - - - - - ranking = 1keywords = gastrointestinal stromal tumour, gastrointestinal stromal, stromal tumour, stromal (Clic here for more details about this article) |
2/8. Primitive neuroectodermal tumors of the uterus: a report of four cases.Four cases of primitive neuroectodermal tumor (PNET) of the uterine corpus are reported, bringing the total number of reported PNETs in this site to seven. The four women were in their seventh decade of life and presented with abnormal vaginal bleeding and, in two cases, an enlarged uterus. The patients underwent total or subtotal abdominal hysterectomy and bilateral salpingo-oophorectomy and, in one patient, pelvic lymphadenectomy. Three patients received postoperative radiation therapy, chemotherapy, or both. Gross examination revealed fleshy polypoid masses filling the endometrial cavity and, in two cases, deeply invading the myometrium. Histologic, immunohistochemical, and, in two cases, ultrastructural examination revealed typical PNETs that exhibited variable degrees of neural, glial, ependymal, and medulloepithelial differentiation. Two PNETs were admixed with other neoplasms: in one case a grade I endometrial adenocarcinoma and in the other a low-grade endometrial stromal sarcoma. The prognosis of the tumors was related to their stage: two patients with stage I tumor were alive with no evidence of disease at 5 and 6 years, whereas two patients with stage III or IV tumor died of tumor at 6 and 12 months. Although it has been suggested that uterine PNETs may be derived from displaced germ cells or implanted fetal tissues, evidence provided by this study, including the advanced ages of the patients and an admixture with neoplasms of unquestioned mullerian origin, suggests a mullerian origin for these tumors in at least some cases.- - - - - - - - - - ranking = 0.0081498044688213keywords = stromal (Clic here for more details about this article) |
3/8. Pancreatic stromal tumor of nerve sheath origin treated by pancreatoduodenectomy.A pancreatic sarcoma of nerve sheath origin is reported in a 28-year-old female patient, who presented with melaena. Preoperative imaging showed an 8.5 cm diameter mass in the head of pancreas. There was bleeding from the papilla of Vater at endoscopy and a highly vascular lesion on arteriography. The patient was submitted to proximal pancreatoduodenectomy and remains symptom-free at 1 year follow-up.- - - - - - - - - - ranking = 0.032599217875285keywords = stromal (Clic here for more details about this article) |
4/8. Gastrointestinal autonomic nerve tumor: a case report with electron microscopic and immunohistochemical analysis and review of the literature.A case of an adolescent girl with metastatic gastric stromal tumor is described. There were three metastatic nodules in the liver at the time of the admission. A subtotal gastrectomy was performed. The tumor had distinctly nodular appearance and was composed of a variety of cells suggestive of smooth muscle differentiation. Electron microscopy revealed cytoplasmic neural processes and densecore neurosecretory granules. immunohistochemistry showed positive neuron-specific enolase, synaptophysin, and chromogranin a in some of the tumor cells. Similar findings in the primary tumor and its liver metastases indicated a primitive neural differentiation and enabled us to classify the lesion as a gastric autonomic nerve tumor. No other tumors that would suggest that the gastric lesion is a part of Carney's triad were detected. The child was treated with chemotherapy but the liver metastases did not change significantly. She is alive with unresectable liver metastases 10 months after the gastrectomy.- - - - - - - - - - ranking = 0.0081498044688213keywords = stromal (Clic here for more details about this article) |
5/8. Gastrointestinal autonomic nerve tumor presenting as high-grade sarcoma. Case report and review of the literature.Gastrointestinal autonomic nerve (GAN) tumors, also known as plexosarcomas, are a rare distinct subtype of the gastrointestinal stromal tumors. These tumors are usually histologically low-grade, epithelioid or spindle-cell neoplasms that can be distinguished from the other gastrointestinal stromal tumors on the basis of their unique ultrastructural features. A 66-year-old female presented with a histologically high-grade sarcoma of the small bowel. Ultrastructural studies showed features of a GAN tumor. The light microscopic and ultrastructural features are described. The tumor cells gave strong, diffuse staining for vimentin and synaptophysin, and weak focal staining for neuron-specific enolase and S100. While usually presenting as low-grade neoplasms on histologic examination, this case demonstrates that GAN tumors should be considered in the differential diagnosis of a histologically high-grade sarcoma of the gastrointestinal tract, especially when evidence of smooth muscle, peripheral nerve sheath, or neuroblastic origin is not forthcoming.- - - - - - - - - - ranking = 0.61696755175303keywords = gastrointestinal stromal, stromal (Clic here for more details about this article) |
6/8. Duodenal stromal tumor with neural differentiation.A 49-year-old woman was admitted to our hospital for detailed evaluation of an abdominal mass. ultrasonography and computed tomography revealed two tumors, one a submucosal lesion of the duodenal second portion and the other a left adrenal tumor. angiography showed that the tumors were hypervascular. Both tumors were removed surgically and examined histologically using hematoxylin-eosin staining. The duodenal tumor (10 x 6 x 5 cm) was initially diagnosed as a schwannoma, but immunohistochemical studies showed that it was S-100 protein negative and neuron-specific enolase positive. Therefore, this tumor was identified as a stromal tumor with neural differentiation. The left adrenal tumor was a nonfunctioning adenoma.- - - - - - - - - - ranking = 0.040749022344106keywords = stromal (Clic here for more details about this article) |
7/8. Gastrointestinal autonomic nerve tumor of the esophagus. A clinicopathologic, immunohistochemical, ultrastructural study of a case and review of the literature.BACKGROUND: Gastrointestinal autonomic nerve (GAN) tumor is a rare tumor that is supposed to originate from the enteric autonomic plexus. The tumor is a subgroup of the gastrointestinal stromal tumor that usually occurs in the stomach and small intestine. methods: An intramural tumor located in the upper third of the esophagus of a 62-year-old Chinese female is reported. The tumor was removed by a three-phased esophagogastrectomy because of its large size. The tumor measured 6.5 cm x 5 cm x 4 cm. Its tissues were sampled, examined by light microscopy, immunohistochemistry, and electron microscopy. RESULTS: The tumor was vaguely encapsulated but had foci of partial infiltration of the capsule. It was comprised of spindle cells with moderate nuclear pleomorphism. The mitotic count was less than 1 per 10 high-power field. The tumor stained positive for vimentin, neuron specific enolase (NSE), and S-100 protein, and was negative for cytokeratins, synaptophysin, chromogranin, neurofilaments, muscle markers, HMB45, and CD34. Ultrastructural study revealed that the tumor had cytoplasmic processes interdigitated in a complex fashion that were held together by primitive junctions but not invested in basal lamina. Many neurosecretory granules and neurotubules were also noted. The diagnosis was GAN tumor of the esophagus. From previous reports, 43 cases (25 males, 18 females) of GAN tumor of other locations have been documented. The tumors were located almost exclusively in the stomach and small intestine; rare cases arose primarily in the retroperitoneum and mesentery. Some of these GAN tumors were observed in patients with Carney's triad (three cases), neurofibromatosis (two cases), and adrenal ganglioneuroma (one case). It appears that the biologic behavior of GAN tumors is aggressive but there are too few reports on which to conclude anything about their prognosis. The tumors are usually large, with low mitotic rate, and are positive for NSE and negative for muscle markers. CONCLUSIONS: To the authors' knowledge, this is the first time that GAN is described in the esophagus. The diagnosis can be made only on the basis of characteristic ultrastructural features.- - - - - - - - - - ranking = 0.30848377587651keywords = gastrointestinal stromal, stromal (Clic here for more details about this article) |
8/8. Gastrointestinal autonomic nerve tumor (plexosarcoma): report of a case with fine needle aspiration biopsy and histologic, immunocytochemical and ultrastructural study.BACKGROUND: gastrointestinal stromal tumors (GISTs) encompass a large group of mesenchymal neoplasms that display common cytologic spindle-shaped morphology on light microscopy. Immunocytochemical and ultrastructural studies can demonstrate several patterns of differentiation. CASE: A 70-year-old male presented with two intraabdominal small bowel masses. The cytopathologic features of a fine needle aspiration biopsy (FNAB) included plump spindle cells in densely populated aggregates or in a fasciculated pattern, without significant pleomorphism. An epithelioid component in a lobular arrangement with abundant, eosinophilic cytoplasm was also noted. The nuclei were vesicular, with a very evident, eosinophilic nucleolus and finely distributed chromatin. Groups of loosely cohesive cells with slender, dendritic-like cytoplasm were evident. Immunocytochemical study of the embedded, fine needle aspirated fragments of the neoplasm demonstrated immunoreactivity for vimentin and neuron-specific enolase. Cytokeratin immunoreactivity or muscular, vascular, neuroendocrine or nerve sheath differentiation failed to be demonstrated. The cytologic and immunocytochemical findings correlated well with the histologic features of the neoplasm. The morphologic diagnosis was confirmed by ultrastructural study. CONCLUSION: FNAB and immunocytochemistry can be valuable in making the correct diagnosis between gastrointestinal stromal tumors.- - - - - - - - - - ranking = 0.31663358034533keywords = gastrointestinal stromal, stromal (Clic here for more details about this article) |