1/18. High mitotic index associated with poor prognosis in gastrointestinal autonomic nerve tumour.AIMS: Three gastrointestinal autonomic nerve tumours (GANT) were characterized by immunohistochemistry and flow cytometry. Two of the three cases occurred in the small intestine, while the third was found in the stomach. Besides the immunohistochemical and ultrastructural description, the aim of this study was to examine the relation between the known and accepted predictive factors (ploidy data, the S-phase fraction, the mitotic and MIB-1 index and the size of the tumour) and the survival of the patients. methods AND RESULTS: The immune profile showed that 3/3 cases were vimentin and NSE, 2/3 were synaptophysin and PGP 9.5 positive, while 1/3 also showed S100 positivity. Ultrastructurally, all the cases had dense core granules, one of them contained skenoid fibres. The flow cytometry revealed diploid dna in all cases, however, significant differences could be seen in the proliferative activity of the individual neoplasms. CONCLUSIONS: In spite of the published data of gastrointestinal stromal tumours (GIST) generally, neither the MIB-1 index and the ploidy data nor the size of the primary tumour helped to predict the clinical progression of the examined GANTs. However, the high proliferative activity (57 mitoses/10 HPF) and the elevated S-phase fraction (24%) was associated with advanced, metastatic and recurring disease in case 3. On the basis of these three cases, high mitotic activity is the most reliable factor in predicting aggressive clinical behaviour.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
2/18. GANT-like gastrointestinal pacemaker cell tumours with oncocytic features.We describe two cases of gastrointestinal stromal tumours with prominent oncocytic features. Both had features consistent with differentiation towards the interstitial cells of cajal (CC). They were composed of nests and bundles of cells with abundant, deeply granular, eosinophilic cytoplasm. Immunohistochemical investigations revealed positivity with c-kit, vimentin and CD34 antibodies in both neoplasms. Ultrastructurally the neoplastic cells showed characteristic features of CC; they had synapse-like structures and dense core cytoplasmic granules. Oncocytic features were confirmed by immunohistochemistry using anti-mitochondrion antibody in both cases and by electron microscopy in one case (case 1). Although the CC are frequently described as mitochondrion-rich cells, oncocytic changes have not previously been reported as a feature of gastrointestinal autonomic nerve tumour (GANT)-like stromal tumours.- - - - - - - - - - ranking = 0.2keywords = nerve (Clic here for more details about this article) |
3/18. Gastrointestinal autonomic nerve tumor: case report and review of the literature.Gastrointestinal autonomic nerve (GAN) tumor is an uncommon specialized form of gastrointestinal stromal tumor (GIST). We report the case of a 46-year-old man affected by this tumor. The neoplasm arose from the sigmoid colon. The patient underwent surgery but eight months later an omental relapse occurred. A second laparotomy was successfully performed and the patient is free of disease at 21 months of follow-up. To our knowledge this is the first case of a large bowel GAN tumor described in the literature.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
4/18. 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 = 1.2keywords = nerve (Clic here for more details about this article) |
5/18. Endoscopic ultrasound-guided fine-needle aspiration cytology of peripheral nerve-sheath tumors.Endoscopic ultrasound (EUS) has allowed for the fine-needle aspiration and diagnosis of many different gastrointestinal neoplasms, including mesenchymal tumors. Although most mesenchymal tumors of the gastrointestinal tract are gastrointestinal stromal tumors (GISTs), other mesenchymal tumors, including neural tumors, do occur. Proper diagnosis and differentiation of these tumors from GISTs are important because of their different prognoses and treatment regimens. We encountered three peripheral nerve-sheath tumors of the gastrointestinal tract aspirated by EUS (two schwannomas and a granular-cell tumor). We report on the endoscopic ultrasound, cytologic, histologic, and immunohistochemical findings of these cases.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
6/18. A patient with a VEGF and endostatin producing gastrointestinal autonomic nerve tumour.Tumour associated neovascularisation has been characterised as chaotic and insufficient. This report details the results of the analysis of angiogenic factors in tumour cyst fluid, pleural fluid, and blood from a patient with a gastrointestinal autonomic nerve tumour. The tumour produced vascular endothelial growth factor and endostatin in large quantities, which may explain the dysfunctional angiogenesis and tendency to bleeding seen in this tumour type.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
7/18. Gastrointestinal autonomic nerve tumors: a surgical point of view.AIM: Gastrointestinal autonomic nerve tumors are uncommon stromal tumors of the intestinal tract. Their histological appearance is similar to that of other gastrointestinal stromal tumors. We report two cases and performed an analysis of the literature by comparing our findings with the available case reports in the medical literature. methods: Two patients were admitted with abdominal tumor masses. One occurred in the stomach with large multiple liver metastases and the second originated in Meckel's diverticulum. The latter site has never been reported previously. Both patients underwent surgery. In one patient gastrectomy, right liver resection and colon transversum resection were performed to achieve aggressive tumor debulking. In the other patient the tumor bearing diverticulum was removed. RESULTS: Postoperative recovery of both patients was uneventful. Histological examination, immunohistochemical analysis and electron microscopy revealed the diagnosis of a gastrointestinal autonomic nerve tumor. The patient with the tumor in Meckel's diverticulum died 6 mo after surgery because of pneumonia. The patient with liver metastases have been alive 13 years after initial tumor diagnosis and 7 years after surgery with no evidence of tumor progression. In light of our results, we performed a thorough comparison with available literature reports. CONCLUSION: Radical surgical resection of gastrointestinal autonomic nerve tumors seems to be the only available curative approach to date, and long term survival is possible even in large metastasized tumors.- - - - - - - - - - ranking = 1.4keywords = nerve (Clic here for more details about this article) |
8/18. Perineurioma of the stomach. A rare spindle cell neoplasm that should be distinguished from gastrointestinal stromal tumor.We report on the first case of benign perineurially differentiated peripheral nerve sheath tumor (perineurioma) presenting as a bleeding gastric mass in a 30-year-old, previously healthy woman with no signs or stigmata of von Recklinghausen's disease or other primary tumor at time of presentation. Gastric resection specimen revealed an ulcerated moderately cellular mesenchymal tumor consisting of elongated wavy spindle cells arranged in a fascicular and sheet-like pattern with focal whorling and occasional alternation of dark staining cellular and light staining hypocellular areas. Tumor cells were strongly immunoreactive for epithelial membrane antigen, CD56 (N-CAM), and vimentin, but were negative for S-100-protein and other lineage-specific epithelial, mesenchymal, hematolymphoid, and reticulo-histiocytic markers. CD117 revealed numerous positive staining mast cells, but the lesional cells were not reacting. We presume that the combined histological and immunohistochemical profiles of this unusual gastric neoplasm are consistent with a diagnosis of perineurioma with a probably benign biological behavior. To our knowledge, this is the first report of gastric perineurioma, an extremely rare mesenchymal lesion that should be considered among the differential diagnoses of gastrointestinal stromal tumor, especially the so-called KIT-negative GIST. Gastrointestinal perineuriomas might be under-recognized, as our case was initially diagnosed as a benign GIST.- - - - - - - - - - ranking = 0.2keywords = nerve (Clic here for more details about this article) |
9/18. Gastrointestinal autonomic nerve tumors. 'Plexosarcomas'.Four plexosarcomas (gastrointestinal autonomic nerve tumors) characterized by light microscopic, immunocytochemical, and ultrastructural examination, including immunoelectron microscopy in one case, are described. The four neoplasms occurred in the small intestine (duodenum, two; jejunum, one; and ileum, one) and they had an aggressive course with either local or distant metastases. The light-microscopic patterns varied from epithelioid and organoid to spindle cells, mimicking endocrine and sarcomatous neoplasms. Ultrastructurally, these tumors exhibited interdigitating cytoplasmic processes that contained scattered aggregates of membrane-bound granules varying in size from 100 to 300 nm intermixed with empty vesicles and numerous diffusely distributed intermediate filaments. Basal lamina covering cell surfaces, attachment plaques, and myofilaments, as expected in smooth-muscle tumors, were not identified, and diffusely distributed membrane-bound granules, as seen in paragangliomas and carcinoid tumors, were also absent. By immunocytochemistry, the tumors were intensely positive for vimentin and neuron-specific enolase and focally positive for neurofilaments and synaptophysin. In addition, three tumors were S100 protein positive and one stained for vasoactive intestinal peptide. Similar positive immunocytochemical reactions were identified in normal enteric plexus. It is essential to recognize plexosarcomas, which are invariably accompanied by aggressive clinical behavior, in spite of a seemingly benign, mitotically inactive light-microscopic appearance in most instances. Ultrastructural examination can readily separate plexosarcomas from paragangliomas and other sarcomatous and endocrine neoplasms.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
10/18. gastrointestinal stromal tumors--a recently defined entity. literature data and personal case report.Stromal tumors of the gastrointestinal tract are controverted tumoral entities which were recently delimited. They were initially identified by immunohistochemical investigation and processing of the gastrointestinal muscular tumors and of the malignant and benign nervous tumors. Those investigations were subsequently amplified by electronmicroscopic studies and observations during the patient's prognostic follow-up. Within these circumstances, monoclonal tumors responsive to smooth muscular antigens (actin, SMA), to polyclonal antigens reacting to S-100, PGP 9.5, NSE and GEAP were identified. Thus, tumors with a nervous origin known as a gastrointestinal nerve tumors or plexosarcomas were differentiated, taking into account the difficulty of their distinguishing from gastrointestinal tumors (GIST) which also shows multinucleated great cells. There were also difficulties in defining bidirectionally differentiated tumors or those nonresponsive to any antigen, all these elements proving their origin from nondifferentiated mesenchymal cells. These data are presented in a personal case report of a 28-year old woman with an acute anteroenteral invagination due to a gastrointestinal stromal tumor, favorably removed by surgery and with a postoperative outcome.- - - - - - - - - - ranking = 0.2keywords = nerve (Clic here for more details about this article) |
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