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)

Endocrine tumors (ET) of the digestive tract (formerly called neuroendocrine tumors) are rare. They are classified into two principal types: gastrointestinal ET's (formerly called carcinoid tumors) which are the most common, and pancreaticoduodenal ET's. Functioning ET's secrete polypeptide hormones which cause characteristic hormonal syndromes. The management of ET is multidisciplinary. Poorly-differentiated ET's have a poor prognosis and are treated by chemotherapy. Surgical excision is the only curative treatment of well-differentiated ET's. The surgical goals are to: 1. prolong survival by resecting the primary tumor and any nodal or hepatic metastases, 2. control the symptoms related to hormonal secretion, 3. prevent or treat local complications. The most common sites of gastrointestinal ET's ( carcinoids) are the appendix and the rectum; these are often small (<1 cm), benign, and discovered fortuitously at the time of appendectomy or colonoscopic removal. Ileal ET's, even if small, are malignant, frequently multiple, and complicated in 30-50% of cases by bowel obstruction, mesenteric invasion, or bleeding. The carcinoid syndrome (consisting of abdominal pain, flushing, diarrhea, hypertension, bronchospasm, and right sided cardiac vegetations) is caused by the hypersecretion of serotonin into the systemic circulation; it occurs in 10% of cases and is usually associated with hepatic metastases. More than half of the cases of pancreatic ET are non-functional. They are usually malignant and of advanced stage at diagnosis presenting as a palpable or obstructing mass or as liver metastases. Insulinoma and gastrinoma (cause of the Zollinger-Ellison syndrome) are the most common functional ET's. 80% are sporadic; in these cases, tumor size, location, and malignant potential determine the type of resection which may vary from a simple enucleation to a formal pancreatectomy. In 10-20% of cases, pancreaticoduodenal ET presents in the setting of multiple endocrine neoplasia (NEM type I), an autosomal-dominant genetic disease with multifocal endocrine involvement of the pituitary, parathyroid, pancreas, and adrenal glands. For insulinoma with NEM-I, enucleation of lesions in the pancreatic head plus a caudal pancreatectomy is the most appropriate procedure. For gastrinoma with NEM-I, the benefit of surgical resection for tumors less than 2-3 cm in size is not clear. The lesions are frequently small, multiple, and widespread and recurrence is frequent after excision. The long-term prognosis is nevertheless fairly good. But the eventual development of liver metastases which are the most common cause of mortality still argues for an aggressive surgical approach in the early stages of the disease.
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PMID:[Surgical treatment of gastric, enteric, and pancreatic endocrine tumors Part 1. Treatment of primary endocrine tumors]. 1614 76

The treacherous and deceptive nature of pheochromocytoma makes it crucial to detect and treat it promptly; otherwise it will almost certainly be fatal from cardiovascular complications or metastases. Hypertension occurring in patients with pheochromocytomas is sustained in about 50% and paroxysmal in the remainder; however, many patients remain normotensive. Hypertension attacks may be precipitated by physical activity, postural changes, anxiety, certain foods or wine, some drugs, operative procedures, etc. Cardinal manifestations are paroxysmal hypertension, headache, palpitations +/- tachycardia, inappropriate sweating; anxiety, tremulousness, pallor (rarely flushing), chest and abdominal pains; nausea and vomiting often occur. Hypercatecholaminemia manifestations are more common and pronounced when paroxysmal hypertension occurs, but persons with familial pheochromocytoma may be asymptomatic. Protean manifestations of pheochromocytoma may simulate many conditions, some of which may have elevated plasma and urine catecholamines and their metabolites. Baro-reflex failure, postural tachycardia syndrome, sleep apnea, carcinoid, renal failure, and pseudopheochromocytoma may be diagnostic challenges. The history, physical examination, biochemical testing (after eliminating interfering drugs, when possible) for plasma and urinary metanephrines can usually establish or exclude presence of pheochromocytomas. Occasionally a clonidine suppression test is needed to differentiate neurogenic from pheochromocytic hypertension. Manifestations suggesting hypercatecholaminemia without hypertension are highly atypical of pheochromocytoma. Pheochromocytoma may present as panic attacks, pre-eclampsia, cardiomyopathy, infection with fever and leucocytosis, diabetes, migraine, shock, Cushing's syndrome, multiple organ failure with lactic acidosis, neurological manifestations, transitory electrocardiogram abnormalities, constipation, intestinal obstruction, visual impairment, convulsions, etc. The key to diagnosis is always to think of pheochromocytoma in the differential diagnosis of hypertension.
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PMID:The protean manifestations of pheochromocytoma. 1924 99

Midgut neuroendocrine tumors (MNETs) are rare, and the primary tumor is usually small and difficult to visualize at imaging. Patients often present late with extensive liver and nodal metastases and may experience symptoms secondary to the release of active substances by the primary tumor, such as serotonin and its metabolites, which have local and systemic effects. Locally, this causes desmoplasia and vascular encasement and may lead to small bowel obstruction and ischemia, with significant morbidity and mortality. Systemically, the release of active substances into the circulation can cause flushing, diarrhea, and abdominal pain (carcinoid syndrome); these substances can be detected in urine and blood serum and used as markers for diagnosis and treatment follow-up. MNETs retain expression of specific peptide receptors such as somatostatin receptors, which will bind to synthetic somatostatin analogs such as octreotide. This feature is useful for functional imaging of patients with MNETs and for peptide receptor radionuclide therapy using somatostatin analogs. Resection of the primary tumor is advocated, even in patients with extensive metastases, because it may prevent development of local complications, can help control systemic symptoms, and has been shown to confer some survival advantage. Computed tomography and functional imaging are used to identify the primary tumor and assess its resectability. The main factors governing resectability are patient comorbidities (eg, carcinoid heart disease), vascular involvement, and desmoplasia.
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PMID:Midgut neuroendocrine tumors: imaging assessment for surgical resection. 2461 88

Neuroendocrine tumors (NET) include a spectrum of malignancies arising from neuroendocrine cells throughout the body. The objective of this clinical investigation of retrospectively and prospectively collected data was to describe the prevalence, demographic data, clinical symptoms and methods of diagnosis of NET and the treatment and long-term follow-up of patients with NET. Data were provided by the participating centers and assessed for consistency by internal reviewers. All the cases were centrally evaluated (when necessary) by the pathologists in our group. The tissue samples were reviewed by hematoxylin and eosin and immunohistochemical staining techniques to confirm the diagnosis of NET. In total, 532 cases were documented: 461 gastroenteropancreatic-NET (GEP-NET) and 71 bronchial NET (BNET). All the tumors were immunohistochemically defined according to the World Health Organization (WHO) and European Neuroendocrine Tumor Society criteria. The most common initial symptoms in GEP-NET were abdominal pain, diarrhea, bowel obstruction, flushing, gastrointestinal bleeding and weight loss. The most common tumor types were carcinoid (58.0%), non-functional pancreatic tumor (23.0%), metastatic NET of unknown primary (16.0%) and functional pancreatic tumor (3.0%). Of the BNET, 89.0% were typical and 11.0% atypical carcinoids. Of the patients with GEP-NET, 59.2% had distant metastasis at diagnosis. The locations of the primary tumors in GEP-NET were the small bowel (26.9%), pancreas (25.2%), colon-rectum (12.4%), appendix (7.6%), stomach (6.9%), esophagus (2.8%), duodenum (2.0%) and unknown primary (16.3%). The histological subtypes based on the WHO classification were well-differentiated NET (20.1%), well-differentiated neuroendocrine carcinomas (66.5%) and poorly differentiated neuroendocrine carcinomas (10.3%). Overall, 67.3% of the patients underwent surgery, 41.2% with curative intent and 26.1% for palliative purposes. The 5-year survival rates were 65.1% (95% confidence interval, 58.0-71.4%) in GEP-NET and 100.0% in typical carcinoid of the lung. This observational, non-interventional, longitudinal study aimed to accumulate relevant information regarding the epidemiology, clinical presentation and current practices in the treatment of NET patients in Argentina, providing insight into regional differences and patterns of care in this heterogeneous disease.
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PMID:Observational study of patients with gastroenteropancreatic and bronchial neuroendocrine tumors in Argentina: Results from the large database of a multidisciplinary group clinical multicenter study. 2505 30

Small intestinal neuroendocrine tumours (SI-NETs) are neoplasms characterized by their ability to secrete biogenic amines and peptides. These cause distinct clinical pathology including carcinoid syndrome, marked by diarrhoea and flushing, as well as fibrosis, notably mesenteric fibrosis. Mesenteric fibrosis often results in significant morbidity by causing intestinal obstruction, oedema and ischaemia. Although advancements have been made to alleviate symptoms of carcinoid syndrome and prolong the survival of patients with SI-NETs, therapeutic options for patients with mesenteric fibrosis are still limited. As improved insight in the complex pathogenesis of mesenteric fibrosis is key to the development of new therapies, we evaluated the literature for known and putative mediators of fibrosis in SI-NETs. In this review, we discuss the tumour microenvironment, growth factors and signalling pathways involved in the complex process of fibrosis development and tumour progression in SI-NETs, in order to elucidate potential new avenues for scientific research and therapies to improve the management of patients suffering from the complications of mesenteric fibrosis.
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PMID:Small intestinal neuroendocrine tumours and fibrosis: an entangled conundrum. 2923 41

Carcinoid tumours have the ability to secrete various peptides and bioamines that lead to carcinoid syndrome manifested as cutaneous flushing, diarrhoea, bronchial constriction and cardiac involvement. The deficiencies of vitamins D and B12 have previously been reported in patients with carcinoid tumours presumably due to chronic diarrhoea associated with the carcinoid syndrome. Herein, we chronicle the case of a patient with opioid use disorder who presented with small bowel obstruction that was found to be caused by a midgut carcinoid tumour. Laboratory studies revealed deficiencies of vitamins D and B12 even though he denied diarrhoea and had no other aetiology of deficiencies of these vitamins. Additionally, this paper presents a review of the published medical literature pertaining to clinical features, diagnostic investigations and treatment of intestinal carcinoid tumours and explores possible explanations for the observed deficiencies in these patients.
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PMID:Vitamin D and vitamin B12 deficiencies in patients with small intestinal carcinoid tumour: is opioid use disorder a confounding factor in the diagnosis? 3087 64

Introduction: Little is known about adult intussusception, but current evidence suggests that malignancy, polyps, and diverticula are usual etiologies. We present a case of adult ileoceccal intussusception secondary to carcinoid tumor. Case Presentation: A 53-year-old African American male presented with hematochezia and non-radiating constant left upper quadrant pain accompanied by nausea and vomiting. CT of the pelvis demonstrated a pathognomic 'target' sign, consistent with ileoceccal intussusception and early small bowel obstruction. Two years prior to this current presentation, the patient had experienced an episode of hematochezia for which he underwent colonoscopy and polypectomy, with subsequent pathology results negative for colon cancer. He denies diarrhea, constipation, weight loss, decreased appetite or skin flushing. Due to persistent symptoms of bowel obstruction, he underwent exploratory laparotomy. During the surgery a white-colored, chalky mass indicative of penetrating tumor was noted 13 cm proximal to the ileocecal valve. An extended right hemi-colectomy followed the discovery of the mass. Pathology showed a well-differentiated neuroendocrine tumor consistent with carcinoid tumor. Evaluation for metastatic disease using 5-HIAA and chromogranin A was unremarkable, and the resection of the right colon carcinoid tumor was felt to be curative. Conclusion: It is uncommon for adults to present with intussusception; in such cases, malignancy should be ruled out as an underlying cause. Carcinoid should be listed among the other secondary causes, which include inflammatory bowel disease, diverticulitis, polyps, scar tissue, adhesions, and lipomas. Abbreviation: CT (Computer tomography), 5-HIAA (5-hydroxyindole acetic acid), NCCN (National Comprehensive Cancer Network).
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PMID:Carcinoid tumor causing ileoceccal intussusception in an adult patient. 3125 72

Carcinoid syndrome (CS) is a paraneoplastic syndrome caused by the release of serotonin and other substances from well-differentiated neuroendocrine tumors (NETs). The hallmark symptoms of carcinoid syndrome are flushing and diarrhea; atypical signs and symptoms can include wheezing, abdominal pain, valvular heart disease, telangiectasias, pellagra, and the complications of mesenteric fibrosis, including ureteral obstruction, bowel obstruction, and bowel ischemia. These symptoms are mediated by the release of serotonin (5-HT), histamine, kallikrein, prostaglandins, and tachykinins. The diagnosis of CS requires these symptoms and corresponding elevations in lab tests. Treatment options include surgery and medical management with somatostatin analogs.
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PMID:Carcinoid Syndrome: A Review. 3225 25