Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UNIPROT:P61278 (somatostatin)
 22,083 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The co-existence of a gangliocytic paraganglioma and a glandular psammomatous carcinoid in the duodenum of a patient with von Recklinghausen's disease and bilateral phaeochromocytomas is reported. The two lesions were considered to be distinctive by light microscopy, electron microscopy and immunocytochemistry. The cells of the glandular carcinoid showed strong cytoplasmic immunoreactivity for somatostatin and contained only scanty intracytoplasmic microfilaments on electron microscopy. In contrast, the endocrine cells of the gangliocytic paraganglioma were positive for pancreatic polypeptide and serotonin, were negative for somatostatin, and contained conspicuous intracytoplasmic aggregates of filaments. The histogenic relationship between the two tumours is discussed. This case strengthens the known association of glandular duodenal somatostatinoma with von Recklinghausen's disease and phaeochromocytoma and, in the light of a previous case report, suggests that von Recklinghausen's disease and gangliocytic paraganglioma may co-exist more commonly than expected.
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PMID:Synchronous duodenal neuroendocrine tumours in von Recklinghausen's disease--a case report of co-existing gangliocytic paraganglioma and somatostatin-rich glandular carcinoid. 289 42

Ten cases of duodenal paraganglioma were studied by conventional histologic and immunocytochemical techniques at both light and electron microscopic levels. Histologically, mixtures of epithelial, ganglion, and spindle cells were seen. In all of the cases immunoreactivity for neuron-specific enolase (NSE) and protein gene product (PGP) 9.5 was seen in each component. Pancreatic polypeptide immunoreactivity was detected in eight cases, mainly in epithelial cells. Somatostatin immunoreactivity was present in epithelial and ganglion cells in nine cases. In seven cases immunoreactivity for neurofilaments, a marker for neurons, was seen in ganglion and spindle cells. However, immunoreactivity for chromogranin, a protein found in endocrine storage granules, was found in only two cases, and the staining was confined to well-granulated epithelial cells. The spindle cells were immunostained only for neuronal markers, NSE and neurofilaments, and the glial marker S-100 protein.
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PMID:Duodenal gangliocytic paragangliomas: a study of 10 cases with immunocytochemical neuroendocrine markers. 353 54

We present the first reported case (to our knowledge) of duodenal gangliocytic paraganglioma (GPG) to be associated with an underlying invasive adenocarcinoma. The patient, a 71-year-old man, presented with epigastric tenderness and was found to have metastatic adenocarcinoma in two regional lymph nodes. Immunohistochemical evaluation of the GPG demonstrated positive staining for gastrin, glial-fibrillary acidic protein, glucagon, neuron-specific enolase, pancreatic polypeptide, S100 protein, somatostatin, and substance P. The clinical, pathologic, and immunohistochemical features of GPG are discussed, with a review of the literature.
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PMID:Gangliocytic paraganglioma associated with duodenal adenocarcinoma. Case report with immunohistochemical evaluation. 380 Jun 4

A patient with catecholamine-producing extra-adrenal paraganglioma was found to have a high concentration of immunoreactive somatostatin (IR-SRIF) in the peripheral blood plasma (47.0 pg/ml, normal; 13.3 +/- 5.3 pg/ml, mean +/- SD). After removal of the tumor, the plasma SRIF level fell within normal range (8.7 pg/ml). In the resected tumor, a considerable amount of IR-SRIF (71.0 ng/g wet weight)( was also detected and SRIF-positive tumor cells were demonstrated by the immunoperoxidase technique. Chromatographic analysis of an extract of the tumor showed the presence of two IR-SRIF components of large molecular size besides a component consistent with authentic tetradecapeptide SRIF (SRIF 14), and one of these had the same molecular size as octacosapeptide SRIF (SRIF 28). This seems to be the first report of a catecholamine-producing extra-adrenal paraganglioma that produces IR-SRIF with molecular heterogeneity.
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PMID:Immunoreactive somatostatin in catecholamine-producing extra-adrenal paraganglioma. 612 12

Requisites for preoperative and intraoperative tumor localization with [111In]diethylenetriaminepentaacetic acid-D-[Phe1]-octreotide scanning were explored in 23 patients with endocrine tumors (15 carcinoids, 4 insulinomas, and single cases of gastrinoma, medullary thyroid carcinoma, aldosteronoma, and paraganglioma). The patients were subjected to Octreoscan single photon emission computed tomographic examination prior to surgery and well counter investigation of nuclide uptake in tumors and normal tissues sampled at surgery. Somatostatin receptor-positive tumors demonstrated efficient nuclide accumulation with mean tumor:blood radioactivity ratios of 180-370 (for carcinoids and insulinoma), compared with tissue:blood ratios of 302 for spleen, 42 for liver, and < 10-15 in other normal tissues (pancreas, small intestine, and mesenteric fat). Inefficient preoperative visualization of lesions was related to inconspicuous size, as for primary intestinal carcinoids, tiny liver metastases, and a single small insulinoma. High background activity, pronounced tumor fibrosis, and meager accumulation of tracer also interfered with visualization. Tumor deposits in organs with low background activity (such as carcinoid mesenteric metastases and endocrine pancreatic tumors) were generally most readily detected. Intraoperative investigations with hand-held gamma detector probes were disturbed by obvious high background activity. These investigations revealed two preoperatively unrecognized primary intestinal carcinoids, which, however, were both palpable during surgery. These studies, therefore, had little impact on the surgical strategy.
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PMID:Human biodistribution of [111In]diethylenetriaminepentaacetic acid-(DTPA)-D-[Phe1]-octreotide and peroperative detection of endocrine tumors. 749 48

Two cases of paraganglioma of the urinary bladder are reported. Their immunohistochemical profiles and the clinical features are compared with other cases in the literature. The three pan-endocrine markers (neuron-specific enolase, synaptophysin and chromogrannin) were positive in both cases. Positivity to other neuropeptides (including the present two cases and those in literature) includes adrenocorticotropic hormone (three out of five cases), calcitonin (two out of nine cases), gastrin (two out of six cases), glial fibrillary acidic protein (one out of five cases), glucagon (two out of six cases), serotonin (five out of nine cases), and somatostatin (four out of eight cases). A previously unmentioned association between paraganglioma of the urinary bladder and carcinoid in the gastrointestinal tract is noted in one of the present cases. This peculiar association highlights the importance of multiplicity of tumours of the neuroendocrine system other than the classical multiple endocrine neoplasia syndromes.
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PMID:Paraganglioma of the urinary bladder: an immunohistochemical study and report of an unusual association with intestinal carcinoid. 810 19

Paragangliomas have neuroendocrine characteristics. We previously described successful in vivo visualization of various tumors of neuroendocrine origin after injection of the radiolabeled somatostatin analogue octreotide. In this study, we report the results of 111In-octreotide scintigraphy in 34 patients referred because of known paragangliomas or in whom a paraganglioma was suspected and compared the results of octreotide scintigraphy with the outcomes of other imaging techniques used in the diagnosis or follow-up of these patients. Fifty of 53 (94%) known localizations in 25 patients with paragangliomas were visualized. In two patients, three localizations were missed during octreotide scintigraphy. Unexpected additional paraganglioma sites, not detected or not investigated with conventional imaging techniques, were found in 9 of 25 patients (36%) with known paragangliomas. In four of them, the supposed tumor localizations were thereafter also demonstrated with other imaging modalities. In eight of nine patients who were referred because of symptoms consistent with paraganglioma or follow-up after surgical removal of a paraganglioma, neither routine imaging nor octreotide scintigraphy revealed any abnormalities indicative of paraganglioma. We conclude that: (1) virtually all paragangliomas can be visualized using in vivo 111In-octreotide scintigraphy and (2) because conventional imaging is usually limited to the site where a paraganglioma is clinically suspected, octreotide scintigraphy, because of the information it provides on potential tumor sites in the whole body, may be useful in detecting multicentricity or metastases in patients with paraganglioma.
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PMID:Octreotide scintigraphy for the detection of paragangliomas. 838 41

Two cases of duodenal gangliocytic paraganglioma were studied by means of immunocytochemical methods using 41 kinds of antibodies. The tumors consisted of three histological types; carcinoid, ganglioneuroma and paraganglioma. Tumors of both cases exhibited immunoreactivity to at least one or as many as three of the following: calcitonin, calcitonin-gene related peptide, endocrine granule constituent, Leu7, neuropeptide Y and basic fibroblast growth factor. In addition, these tumors were also immunopositive for neuron specific enolase, S-100 protein, neurofilament protein, pancreatic polypeptide, chromogranin A, somatostatin, leuenkephalin, substance P and vasoactive intestinal peptide, as has been described in previous reports. In one case, tumor cells were immunopositive for adrenocorticotropin, bombesin, gastrin releasing peptide, myelin basic protein, neuroendocrine marker and tyrosine hydroxylase. Moreover, paraganglioma cells of tumors showed both argyrophilia and argentaffinity. These results suggest that duodenal gangliocytic paraganglioma may originate from embryonic neuroinsular complex.
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PMID:Two cases of duodenal gangliocytic paraganglioma: immunocytochemical characteristics. 882 94

Chemodectomas, or glomus tumours, are unusual head and neck paragangliomas. A non-invasive imaging technique, 123I-metaiodobenzylguanidine (123I-MIBG) scintigraphy, has long been used for the diagnosis of all types of paraganglioma. The aim of this study was to evaluate and compare classic 123I-MIBG scintigraphy with the more recent 111In-pentetreotide scintigraphy in the diagnosis and location of chemodectomas. We performed 123I-MIBG and 111In-pentetreotide scintigraphy in eight patients (7 females, 1 male) with histologically or radiologically confirmed chemodectomas (five carotid body and three jugulotympanic chemodectomas). 123I-MIBG uptake was visualized in four patients on planar views and SPET images (sensitivity 50%); uptake was low in three patients. Using 111In-pentetreotide scintigraphy, all chemodectomas in eight patients were visualized (sensitivity 100%) and 111In-pentetreotide uptake was high in all cases. In conclusion, our results indicate that 111In-pentetreotide scintigraphy is superior to 123I-MIBG scintigraphy in the diagnosis and location of chemodectomas. In-pentetreotide or 123I-MIBG uptake suggests a neuroendocrine origin, providing important functional information in the diagnosis of chemodectomas. Moreover, 111In-pentetreotide scintigraphy permits a good classification of patients with or without somatostatin receptors in the chemodectoma in the application of pharmacological therapy with somatostatin analogues to inoperable tumours. The main therapeutic action of cold somatostatin analogues is to inhibit hormonal hypersecretion in different neuroendocrine tumours. In chemodectomas, however, the most important effect of somatostatin analogues is to reduce tumour volume or inhibit growth progression.
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PMID:111In-pentetreotide scintigraphy is superior to 123I-MIBG scintigraphy in the diagnosis and location of chemodectoma. 975 27

Paragangliomas are neuroendocrine tumors located primarily in the head and neck region, but they can also occur at other sites. Confirming the preoperative diagnosis and detecting synchronous tumors may be difficult in some patients. Octreotide is a somatostatin analog that, when coupled to a radioisotope, produces a scintigraphic image of tumors expressing somatostatin type 2 receptors. Paragangliomas, like many neuroendocrine tumors, have been found to have a high density of somatostatin type 2 receptors on the cell surface. This study compared the results of preoperative octreotide scintigraphy with the histopathology in 21 patients who underwent surgery for presumed head and neck paragangliomas. Octreotide scan findings were positive in 16 patients with paragangliomas and negative in 3 patients with other pathology. One false-positive and 1 false-negative result were obtained. Thus, this test had an accuracy of 90%, a sensitivity of 94%, and a specificity of 75%. Previously unidentified synchronous tumors were identified in 5 patients. On the basis of this series of patients, octreotide scintigraphy appears to be a reliable test to detect paragangliomas and may be quite helpful in preoperative planning.
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PMID:Octreotide scintigraphy for the detection of paragangliomas. 1069 10


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