Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0016382 (flushing)
 6,387 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We report a case of metastatic thymic neoplasm occurring in a 62-year-old Caucasian man. The tumor was polymorphic, with intimately admixed carcinoid, sarcomatous and undifferentiated components. Sarcomatous cells were spindle-shaped and immunoreactive for vimentin, actin and desmin. Carcinoid cells reacted for cytokeratin as well as with a panel of general neuroendocrine markers, including chromogranins A and B, synaptophysin, protein gene product (PGP) 9.5, neuron specific enolase (NSE), Leu 7 and Grimelius' silver. The endocrine nature of the latter cells was confirmed by ultrastructural evidence of abundant electron-dense granules 100 to 300 nm in size. Among the various hormones investigated immunohistochemically only the alpha chain of human chorionic gonadotropin (alpha-hCG) was detected in tumor cells. The neoplasm recurred after surgical resection and caused a typical carcinoid syndrome with skin flushing and increased urinary 5-hydroxyindolacetic acid (5-HIAA). The diagnosis of multidirectional thymic carcinoma with two lines of differentiation, of carcinoid and sarcomatoid type, was made.
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PMID:Multidirectional carcinoma of the thymus with neuroendocrine and sarcomatoid components and carcinoid syndrome. 206 96

Gastrointestinal (GI) carcinoids are ill-understood, enigmatic malignancies, which, although slow growing compared with adenocarcinomas, can behave aggressively. Carcinoids are classified based on organ site and cell of origin and occur most frequently in the GI (67%) where they are most common in small intestine (25%), appendix (12%), and rectum (14%). Local manifestations--mass, bleeding, obstruction, or perforation--reflect invasion or tumor-induced fibrosis and often result in incidental detection at emergency surgery. Symptoms are protean (flushing, sweating, diarrhea, bronchospasm), usually misdiagnosed, and reflect secretion of diverse amines and peptides. Biochemical diagnosis is established by elevation of plasma chromogranin A (CgA), serotonin, or urinary 5-hydroxyindoleacetic acid (5-HIAA), while topographic localization is by Octreoscan, computerized axial tomography (CAT) scan, or endoscopy/ultrasound. Histological identification is confirmed by CgA and synaptophysin immunohistochemistry. Primary therapy is surgical excision to avert local manifestations and decrease hormone secretion. Hepatic metastases may be amenable to cytoreduction, radiofrequency ablation, embolization alone, or with cytotoxics. Hepatic transplantation may rarely be beneficial. Chemotherapy and radiotherapy have minimal efficacy and substantially decrease quality of life. Intravenously administered receptor-targeted radiolabeled somatostatin analogs are of use in disseminated disease. Local endoscopic excision for gastric (type I and II) and rectal carcinoids may be adequate. Somatostatin analogues provide the most effective symptomatic therapy, although interferon has some utility. Overall 5-year survival for carcinoids of the appendix is 98%, gastric (types I/II) is 81%, rectum is 87%, small intestinal is 60%, colonic carcinoids is 62%, and gastric type III/IV is 33%.
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PMID:Current status of gastrointestinal carcinoids. 1588 61

Neuroendocrine carcinoma is a type of neuroendocrine system neoplasm that occurs in organisms in which amine precursor uptake and decarboxylation (APUD) cells are uncommon. In the human body, the most common sites of neuroendocrine carcinoma are the gastrointestinal tract and the lungs. From a histologic standpoint, these tumors are identified by their positive reaction to silver stains and specific markers for neuroendocrine tissues, such as chromogranin A, synaptophysin, and neuron-specific enolase. In addition to local symptoms, these tumors may cause flushing, wheezing, and episodic diarrhea secondary to the release of vasoactive substances. Our medline search encompassing the 20-year period prior to 2003 found few cases of this type of tumor in the paranasal sinuses and nasal cavities and only 1 case of an isolated neuroendocrine carcinoma in the nasopharynx. We describe another case of an isolated nasopharyngeal neuroendocrine carcinoma, which we observed in a 74-year-old woman. We review the clinical presentation and the surgical and systemic treatment.
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PMID:Nasopharyngeal neuroendocrine carcinoma: a case report. 1910 29

Neuroendocrine tumours (NETs) are increasing in both incidence and prevalence and, as a group, are more prevalent than either gastric, pancreatic, oesophageal or hepatobiliary adenocarcinomas, or any two of these cancers combined. Clinical awareness of the protean and intermittent symptoms of NETs (eg, sweating, flushing, diarrhoea, and bronchospasm) is critical for timely diagnosis; however, the classical carcinoid syndrome is relatively uncommon. The most useful diagnostic test for gastrointestinal NETs is measurement of plasma chromogranin A (CgA) levels. Disease extent is assessed by both anatomical imaging, and nuclear imaging with radiolabelled somatostatin analogues. Pathological evaluation comprises tumour-node-metastasis classification, a minimum pathological dataset, CgA and synaptophysin immunostaining, as well as mitotic count or Ki-67 index (a marker of cell proliferation) to define grading. Resection of the primary lesion and as much metastatic disease as possible increases the efficacy of medical therapy. Other management strategies include hepatic embolisation and peptide receptor radionuclide therapy. Patients with tumours expressing somatostatin receptors should be treated with somatostatin analogues. Depending on the tumour grade, other effective agents include cytotoxics, tyrosine kinase inhibitors, and antiangiogenics. The overarching requirement for best management of patients with NETs is to ensure that they have ready access to experienced multidisciplinary clinician groups located within centres of appropriate subspecialty expertise.
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PMID:Gastrointestinal neuroendocrine (carcinoid) tumours: current diagnosis and management. 2061 15