Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UNIPROT:P01275 (glucagon)
 26,492 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The histogenesis of alveolar soft part sarcoma (ASPS) is a subject of continued debate. Although many recent reports suggest a muscle origin, others advocate a neuroendocrine derivation. A tumor in the chest wall of a 16-year-old woman was diagnosed and treated as ASPS. The light microscopic, electron microscopic, and immunohistochemical findings showed features of both ASPS and paraganglioma. In addition, this lesion was positive for antibody to glucagon, a characteristic of neither ASPS nor paraganglioma, although seen in a few gangliocytic paragangliomas. This case demonstrates the need for continued inquiry into the histogenesis of ASPS.
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PMID:An unusual organoid tumor. Alveolar soft part sarcoma or paraganglioma? 200 2

We report the immunohistochemical and ultrastructural features of three duodenal gangliocytic paragangliomas and compare them with duodenal carcinoid, extra-adrenal paraganglioma, pheochromocytoma, and ganglioneuroma. The gangliocytic paraganglioma is characterized by polygonal or columnar epithelial cells, ganglion cells, and spindle cells. The epithelial cells stained for neurofilament, neuron-specific enolase, pancreatic polypeptide, and somatostatin in three cases; leu-enkephalin, molluskan cardioexcitatory peptide, and vasoactive intestinal peptide in two; and glucagon and insulin in one case each. The ganglion cells were positive for leu-enkephalin, neurofilament, neuron-specific enolase, pancreatic polypeptide, and somatostatin in three cases, and glucagon in one. The spindle cells stained for neurofilament, neuron-specific enolase, and S-100 protein. Although there was some overlap in immunoreactivity between the gangliocytic paraganglioma and the other tumors examined, our data indicate that the gangliocytic paraganglioma is a distinctive lesion. We propose that it is a hyperplastic or neoplastic proliferation of 1) endodermally derived epithelial cells originating from the ventral primordium of the pancreas, 2) neuroectodermal ganglion cells, and 3) neuroectodermal spindle cells (Schwann cells).
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PMID:Duodenal gangliocytic paraganglioma. An immunohistochemical and ultrastructural study and a hypothesis concerning its origin. 257 47

A juxtapapillary duodenal polyp (measuring 1.5 x 1.0 x 1.2 cm) was removed endoscopically in a 57-year-old man who had had recurrent intestinal bleedings. Histological examination revealed a typical duodenal gangliocytic paraganglioma with carcinoid and paraganglioma-like components, as well as neuroid portions with ganglion cells. Various neuroendocrine markers (neuron-specific enolase, chromogranin, protein-gene product 9.5) were demonstrated immunohistochemically. Tests for pancreatic polypeptide, substance P, enkephalin, cholecystokinin, glucagon and S-100 proteins, were all positive.
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PMID:[Juxtapapillary gangliocytic paraganglioma of the duodenum]. 270 35

A nonchromaffin paraganglioma was found in the periglandular connective tissue of the glandula suprarenalis of a sheep-dog bastard and characterized by histological and immunohistochemical techniques. Insulin-, glucagon- and gastrin/cholecystokinin-like material was revealed within the cells of the tumour. Possible functional consequences of these findings are discussed.
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PMID:A polypeptide immunoreactive nonchromaffin paraganglioma in the periglandular connective tissue of glandula suprarenalis of a dog. A case report. 298 19

Carcinoid tumors of the middle ear are rare, with only three previously reported cases. The authors report the light and electron microscopic and immunohistochemical features of two carcinoid tumors that occurred in a 34-year-old female and a 21-year-old male. Both presented with unilateral hearing loss. By light microscopic examination, both were characterized by trabecula of tall columnar cells with basal nuclei and no mitotic activity. Electron microscopic examination demonstrated large numbers of pleomorphic neurosecretory granules, perinuclear aggregates of intermediate filaments, cell junctions, and surface microvillous processes. Some cells contained intermediate filaments forming tonofilaments and lacked secretory granules. These cells stained for cytokeratin by immunoperoxidase and separated the neuroendocrine cells from the underlying basal lamina. The cells in this tumor stained for the molluscan cardioexcitatory peptide. Cells in both tumors also stained for pancreatic polypeptide. Neither case stained for lysozyme, insulin, glucagon, somatastatin, gastrin, substance P, thyroid-stimulating hormone, adrenocorticotropic hormone, Met-enkephalin, Leu-enkephalin, neuropeptide Y, peptide YY, neurotensin, Bombesin, serotonin, neuron-specific enolose, glial and neural filaments, S-100 protein, cholecystokinin, beta-endorphin, beta-human chorionic gonadotropin, luteinizing hormone/follicle-stimulating hormone, vasoactive intestinal polypeptide, prolactin or calcitonin. Carcinoid tumor of the middle ear can be distinguished from paraganglioma and middle ear adenoma.
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PMID:Carcinoid tumors of the middle ear. 357 33

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

We treated a hypertensive patient with recurrent pheochromocytoma (paraganglioma) associated with glucagonoma. No clinical symptom of glucagonoma was found and it could be detected only by a slight elevation of plasma immunoreactive glucagon (IRG) while the extirpated pancreatic tumor contained much IRG. This case could not be classified as either multiple endocrine neoplasia (MEN) type I or type II.
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PMID:Recurrent pheochromocytoma associated with glucagonoma. A case report. 613 77

Pheochromocytoma scanning using 131J-meta-benzylguanidine was done in one patient with metastasizing paraganglioma, one patient with multiple endocrine neoplasia type IIb. No activity of tumour tissue could be demonstrated in the patient with metastasizing paraganglioma, whereas the pheochromocytoma could be clearly defined in the patient with multiple endocrine neoplasia type IIa. The female with multiple endocrine neoplasia type IIb showed a suspect space-occupying lesion of the left adrenal using computed tomography. Pheochromocytoma could be excluded by 131J-benzylguanidine scanning, selective estimation of catecholamines in adrenal venous blood as well as the glucagon stimulation and clonidine suppression.
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PMID:[Imaging adrenergic tumours with 131J-meta-benzylguanidine]. 711 46

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

This article gives an overview of the established radionuclide therapies for endocrine-related cancer that already have market authorization or are currently under evaluation in clinical trials. Radioiodine therapy is still the gold standard for differentiated iodine-avid thyroid cancer. In patients with bone and lung metastases (near) total remission is seen in approximately 50% and the 15-year survival rate for these patients is approximately 90%. In contrast to the USA, meta-iodobenzylguanidine (MIBG) therapy has market approval in Europe. According to the current literature, in the setting of advanced stage neuroblastoma and malignant pheochromocytoma or paraganglioma, radiological remission can be achieved in >30% and symptom control in almost 80% of the treated patients. Somatostatin receptor targeted radionuclide therapies (e.g. with DOTATATE or DOTATOC) demonstrated promising results in phase 2 trials, reporting progression-free survival in the range of 24-36 months. A first phase 3 pivotal trial for intestinal carcinoids is currently recruiting and another trial for pancreatic neuroendocrine tumors is planned. Radiopharmaceuticals based on glucagon-like peptide 1 (GLP1) or minigastrins are in the early evaluation stage for application in the treatment of insulinomas and medullary thyroid cancer. In general, radiopharmaceutical therapy belongs to the group of so-called theranostics which means that therapy is tailored for individual patients based on molecular imaging diagnostics to stratify target positive or target negative tumor phenotypes.
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PMID:[Radionuclide therapy of endocrine-related cancer]. 2526 25


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