Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UNIPROT:P01189 (beta-endorphin)
 21,003 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

An autopsy case of a 9-year-old Japanese girl revealed a carcinoid tumor originating in the duodenum and hyperplasia of the multiple endocrine organs as manifested by ectopic ACTH syndrome, carcinoid syndrome and giantism. The tumor cells were positive for histochemical argyrophile reaction and two types of secretory granules were identified by electron microscopy. Biochemical assay revealed the production of ACTH and beta-MSH by the tumor cells. Other changes of multiple endocrine organs included acidophil dominant hyperplasia of the pituitary, diffuse hyperplasia of the thyroid, chief cell hyperplasia of the parathyroid, hyperplasia of the islets of Langerhans and the adrenal cortex. This case was considered to be a type of multiple endocrine adenomatosis including carcinoid tumor. The relationship between the carcinoid tumor and multiple endocrine adenomatosis was discussed.
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PMID:Ectopic ACTH-MSH producing carcinoid tumor with multiple endocrine hyperplasia in a child. 21 30

An ovarian strumal carcinoid which synthesized peptide hormones, but did not induce the carcinoid syndrome, was analysed histochemically, immunohistochemically and ultrastructurally. Dot-immunobinding assays were performed in order to determine the endocrine gene expression. The amylase resistant colloid was found to be PAS-positive in the follicular portions of the tumour. Carcinoid cells showed Grimelius positive argyophilic granules in the subnuclear position. The Fontana-Masson argentaffin reaction was negative. Immunohistochemistry for adrenocorticotropic hormone (ACTH) revealed strong reactivity in the follicular areas of the carcinoid. The immunoreactivity for somatotropic release inhibiting factor (SRIF) was found positive in the trabecular portion of the carcinoid tumour, thyroglobulin in the follicles. Neuron-specific enolase, protein S-100 A/B, synaptophysin and chromogranin A evoked weak cytoplasmic immunostaining of the tumor cells. Dot-immunobinding assays substantiated these immunohistochemical results, except for the thermolabile protein S-100 A/B. Electron microscopy of tumor cells showed numerous electron-dense cytoplasmic granules, 250 to 350 nm in diameter, both in follicular and trabecular areas of the tumor. Plasma levels of tumor-associated ACTH, SRIF and thyroglobulin were measured by radioimmunoassay and were found to be within the normal range.
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PMID:Immunochemical and ultrastructural studies of an ovarian strumal carcinoid. 198 59

Eight ovarian heterologous Sertoli-Leydig cell tumors containing gastrointestinal-type cells, including two tumors that contained carcinoids, were stained for argyrophilia and argentaffinity; in addition, these specimens were stained by immunohistocytochemical techniques for the demonstration of chromogranin, serotonin, and a variety of peptide hormones. Intestinal- and gastric-type epithelial and carcinoid cells within the tumors were focally argyrophilic and chromogranin-positive, but only intestinal-type epithelial and carcinoid cells contained argentaffin granules, serotonin, and corticotropin. Somatostatin, gastrin, neurotensin, and glucagon were demonstrated additionally in varying numbers of specimens containing intestinal-type epithelium and carcinoid, and somatostatin was present in gastric-type epithelium in one case. Staining for calcitonin and insulin was negative. Despite the frequent identification of serotonin and peptide hormones in the tumors in the present series, evidence of the carcinoid syndrome or syndromes associated with peptide hormone excess was lacking on review of the patients' records.
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PMID:Ovarian heterologous Sertoli-Leydig cell tumors with gastrointestinal-type epithelium. An immunohistochemical analysis. 375 27

Twenty-five endocrine tumors of the rectum (rectal carcinoids) were examined immunohistochemically for various pancreatic and gut neurohormonal polypeptides. Twenty-one of the tumors were found to contain cells displaying pancreatic polypeptide (PP), glucagon, somatostatin, insulin, substance P, enkephalin or beta-endorphin immunoreactivity. At least 11 of the tumors contained more than one peptide hormone. In some of the tumors PP cells made up the major cell population, in others the glucagon cells constituted the majority. Only four of the tumors contained 5-hydroxytryptamine. Rectal endocrine tumors seem unique among gut endocrine tumors in that they may store immunoreactive enkephalin, beta-endorphin and even insulin. None of the patients displayed the carcinoid syndrome; symptoms were usually vague and uncharacteristic. In many cases the tumor was found at routine examination.
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PMID:Immunohistochemical evidence of peptide hormones in endocrine tumors of the rectum. 617 Apr 21

Neuroendocrine tumors are usually slow-growing tumors. Many of these are capable of secreting peptide hormones or biogenic amines that may lead to endocrine syndromes. Nonfunctioning tumors can either secrete no hormones at all, or secrete hormones not giving rise to endocrine symptoms, such as chromogranin A, chromogranin B or pancreatic polypeptide. Chromogranin A is produced by the majority of endocrine tumors, both functioning and nonfunctioning, and is the best available marker for diagnosis, follow-up and treatment monitoring of patients with differentiated neuroendocrine tumors. Examples of endocrine syndromes are classical carcinoid syndrome caused by serotonin (measured in the urine as its metabolite 5-HIAA), insulinoma syndrome caused by insulin or proinsulin, Zollinger-Ellison syndrome resulting from gastrin secretion, glucagonoma syndrome caused by glucagon, WDHA syndrome caused by vasoactive intestinal peptide, or Cushing's syndrome resulting from ectopic production of adrenocorticotropic hormone or corticotropin-releasing hormone. In case there is uncertainty about the diagnosis, specific tests can be applied, such as the secretin test for diagnosis of gastrinomas and the 72-hour fast for diagnosis of an insulinoma. In patients with suspicion of an inherited syndrome, such as multiple endocrine neoplasia (MEN) 1 and MEN2 syndromes, genetic testing is indicated.
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PMID:Biochemical Testing in Patients with Neuroendocrine Tumors. 2630 2

Pancreatic neuroendocrine tumors (pNETs) constitute a heterogenous group of malignancies with varying clinical presentation, tumor biology and prognosis. The incidence of pNETs has steadily increased during the last decades with an estimated incidence 2012 of 4.8/100,000. Recent whole genome sequencing of pNETs has demonstrated mutations in the DNA repair genes MUTYH and point mutations and gene fusions in four main pathways from chromatin remodeling, DNA damage repair, activation of mechanistic target of rapamycin (mTOR) signaling and the telomere maintenance. This new information will be the foundation for new therapies in the near future for malignant pNETs. The functioning pNETs constitute about 30-40% of all pNETs displaying nine different clinical syndromes: insulinoma, Zollinger-Ellison, Verner-Morrison, glucagonoma, somatostatinomas, ectopic adrenocorticotropic hormone (ACTH) and parathyroid hormone related peptide (PTH-rP) syndromes. Single patients might also present carcinoid syndrome. The diagnostic work-up include histopathology with the new WHO 2017 Classification, biomarkers (CgA, NSE), radiology and molecular imaging including CT-scan, magnetic resonance imaging (MRI), ultrasound and PET-scan. A cornerstone in the treatment of pNETs is surgery which is rarely curative but can reduce the clinical symptoms by debulking which also include radiofrequency ablation, embolization of liver metastases. Medical treatment includes chemotherapy and the targeted agents such as everolimus, sunitinib and peptide receptor radiotherapy (PRRT). Somatostatin analogs has for the last decades been the main stay for management for clinical symptoms related to functioning pNETs and is often combined with new targeted agents as well as chemotherapy. Long-term management of functioning pNETs need a combination of different procedures, surgery, local ablation, targeted agents and somatostatin analogs. Future therapies might be based on the recent advances in molecular genetics and tumor biology.
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PMID:Management of functional neuroendocrine tumors of the pancreas. 2962 16