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Query: UMLS:C0016382 (flushing)
 6,387 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Carcinoid tumours offer a diagnostic and therapeutic challenge. Although new biochemical markers and improved methods for tumour detection, including PET and somatostatin receptor scintigraphy, have been developed during the last two decades many patients are still diagnosed at late stages of the disease. This is supported by the fact that the age of diagnosis is about the same today as it was 10 years ago. It is our opinion that plasma chromogranin A levels should be be determined in all patients which are investigated because of symptoms that might be connected to a neuroendocrine tumour. In cases with flushing or diarrhoea, U-5-HIAA should also be determined and these two tumour markers are enough to diagnose most patients with midgut carcinoid tumours. In patients with foregut or hindgut tumours other specific hormones should be included. For the localization procedure conventional radiological techniques including CT, MRI and ultrasound investigations should be supplemented with somatostatin receptor scintigraphy. Endoscopic ultrasound investigations might in the future be relevant for diagnosis of duodenal carcinoids, whereas gastric and rectal carcinoids are diagnosed by endoscopy. A combination of more aggressive surgery combined with medical treatment such as somatostatin analogues and alpha-interferon has significantly increased the survival rates in patients with classical midgut carcinoid tumours. Metastatic foregut and hindgut tumours are still a therapeutic challenge and it is important in the future to classify all carcinoid tumours based on specific tumour biology patterns. Such a tumour biology based treatment is a prerequisite for a more individually based therapy in the future.
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PMID:Carcinoid tumours. 911 14

Although chromogranin A (CgA) is a recognized marker of neuroendocrine tumours, little is known about the distribution of the CgA-derived peptides, vasostatin (VST) I or II, in these tumours. Rabbit polyclonal antiserum was raised to a fragment of VST I and used to immunostain sections (5 microns) of wax-embedded tumour tissue. Immunoreactivity (IR) was detected using swine anti-rabbit fluorescein secondary antibody and sections were viewed by fluorescence microscopy. Of 24 tumours from patients with lung carcinoids, one was weakly positive, while 23 of 26 ileal carcinoid tumours were immunoreactive. Metastatic deposits from patients with ileal carcinoids also tended to be immunoreactive (9/10). The difference in IR between lung and ileal carcinoid primary tumours did not appear to be related to the metastatic potential, since appendiceal tumours, which seldom metastasize, also tended to be immunoreactive (4/6) for VST I. The strongest IR was recorded in two patients with flushing as a result of ileal carcinoids; five other 'flushers' with ileal carcinoids were also immunopositive for VST I-like IR. By contrast, patients with flushing as a result of lung carcinoids were immunonegative for VST. In conclusion, VST I-like IR may assist in the identification of a secondary deposit from an unknown primary site.
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PMID:Immunostaining for vasostatin I distinguishes between ileal and lung carcinoids. 1039 86

Neuroendocrine gastroenteropancreatic tumors are rather rare neoplasms with an incidence of 1-2 cases per 100,000 people. They show rather varying tumor biology and present sometimes distinct clinical symptoms such as flushing, diarrhoea, hypoglycemia and gastric ulcers. The biochemical diagnosis is today significantly improved by the introduction of chromogranin A as a general tumor marker, which is also useful in histopathology. Today the localization procedures include somatostatin receptor scintigraphy as the primary investigation together with CT or ultrasonography. The basis for treatment of neuroendocrine GEP tumors is not only a curative intent but merely amelioration of clinical symptoms, abrogation of tumor growth, maintaining and improvement of quality of life. Surgery has always to be considered in the treatment of neuroendocrine GEP tumors. It can be performed whenever during the course of the disease but it may be more productive in earlier stages. Liver dearterialization procedures can furthermore reduce the tumor masses in liver together with laser treatment or radiofrequency therapy. The medical treatment includes cytotoxic agents, alpha interferons and somatostatin analogues. Somatostatin analogues will always be combined with the other two alternatives to reduce clinical symptoms. Chemotherapy is particularly useful for patients with more aggressive tumors with high proliferation capacity, whereas alpha interferon is beneficial in classical midgut carcinoids with low proliferation capacity. Quite recently somatostatin based radioactive tumor targeted treatment has evolved with preliminary promising data but further studies are needed to deliniate its future role in the treatment of neuroendocrine tumors in patients.
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PMID:State of the art and future prospects in the management of neuroendocrine tumors. 1093 97

Gastroenteropancreatic (GEP) neoplasms originate from any of the various cell types belonging to the neuroendocrine system. A general characteristic of GEP endocrine tumours is that the vast majority produce and secrete a multitude of peptide hormones and amines. Many patients with malignant metastasising tumours present clinical symptoms related to hormone hyperproduction. These include the so-called carcinoid syndrome, characterised by flushing, diarrhoea, wheezing and right heart disease, which is predominantly associated with the serotonin- and tachykinins-producing carcinoids of the midgut. Several types of syndrome associated with GEP endocrine tumors are caused by overproduction of a specific hormone. For instance, the well-known Zollinger-Ellison syndrome is gastrin-mediated. The so-called 'insulinoma syndrome' depends on excessive production of insulin and proinsulin, resulting in hypoglycemia. The 'glucagonoma syndrome' is characterised by necrolytic migratory erythema, diabetes and diarrhoea. The Verner-Morrison syndrome, which is brought about by high circulating levels of vasointestinal peptide (VIP). produces severe secretory diarrhoea. Finally the 'somatostatinoma syndrome' involves gallbladder dysfunction and gallstones, diarrhoea with or without steatorrhea, and impaired glucose tolerance. The biochemical diagnosis of endocrine digestive tumors is based on general and specific markers. The best general markers are chromogranin A (CgA) and pancreatic polypeptide (PP). Specific markers for endocrine tumors include insulin, gastrin, glucagon, vaso intestinal polypeptide (VIP), somatostatin and the primary cathabolic product of serotonin, 5-hydroxyndoleacetic acid (5-HIAA). Localisation procedures commonly applied, in the diagnosis of endocrine tumours include ultrasound (US), computed tomography (CT) and somatostatin receptor scintigraphy (SRS).
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PMID:Epidemiology, clinical features and diagnosis of gastroenteropancreatic endocrine tumours. 1176 60

We report a potential pitfall of 123I-metaiodobenzylguanine (MIBG) scan. Magnetic resonance imaging performed for other reasons, showed 2.5 cm tumor in the left adrenal gland. On questioning patient had episodic palpitations, flushing and hypertension suggestive of pheochromocytoma. Urinary metanephrine level was of borderline value but serum chromogranin A level was clearly elevated. 123I-MIBG scan showed accumulation of the tracer in the upper left abdomen and the finding was suspected to be intra-adrenal pheochromocytoma. During operation two separate tumors, adrenocortical adenoma in the left adrenal gland, and another smaller, extra-adrenal paraganglioma locating very close to the adenoma, were found. Thus the positive MIBG finding was caused by a paraganglioma with the concurrent presentation of nonfunctioning adrenocortical adenoma.
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PMID:A pitfall of metaiodobenzylguanidine scan: paraganglioma in close proximity to adrenocortical adenoma. 1295 37

Recent publications indicate that life may be prolonged by surgical debulking of neuroendocrine tumors. A minimum 90% reduction of liver metastases has been suggested to alleviate symptoms of the carcinoid. We have used the tumor marker chromogranin A (CgA) to assess hepatic resection in patients with neuroendocrine metastatic tumor disease. Since 1998, seven patients (3 men) of median age 73 years (range 64-84 years) with carcinoid primary tumors in the ileum who had solitary (n = 2) or multiple (n = 5) liver metastases underwent hepatic resections. Two patients had synchronous small intestinal and liver resections; the rest had deferred hepatic resections after intestinal resection. Hormonal manifestations in the form of loose stools or diarrhea or flushing were observed in five patients, and five had abdominal symptoms from partial obstruction of the small bowel. The resection was deemed radical in five patients. Two patients with non-radical resection needed postoperative octreotide treatment, and symptoms were alleviated or improved in the others. All seven patients are alive with an observation period from 6 to 64 months (median 36 months). Median CgA (normal < 30 ng/ml) was 292 ng/ml (range 79-14,000 ng/ml) before liver surgery. Postoperatively, CgA became normal in three of the radically resected patients, whereas in two others, it decreased to a lowest median level of 79 ng/ml (range 52-105 ng/ml). In two palliatively resected patients, one had a near normalization to 65 ng/ml, and the last patient had a reduction from 14,000 to 2400 ng/ml following debulking surgery. A similar postoperative reduction was noted for 24 hr urinary 5-HIAA excretion. Postoperative octreotide scintigraphy suggested residual hepatic or extrahepatic tumors in three of the patients thought radically resected, whereas two had no clear sign of disease corresponding to a normal CgA value. The CgA values, however, reflected the extent of positive scintigraphy findings. Serum CgA levels monitored the extent and short-term course of the disease and corresponded well with scintigraphy findings and 5-HIAA excretion, but prolonged follow-up in more patients may be necessary before decisive conclusions are allowed to be drawn.
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PMID:Chromogranin A, a marker of the therapeutic success of resection of neuroendocrine liver metastases: preliminary report. 1559 63

This 6-month, open, non-controlled, multicenter, dose-titration study evaluated the efficacy and safety of 28-day prolonged-release (PR) lanreotide in the treatment of carcinoid syndrome. Eligible patients had a carcinoid tumor with > or =3 stools/day and/or > or =1 moderate/severe flushing episodes/day. Six treatments of 28-day PR lanreotide were administered by deep subcutaneous injection. The dose for the first two injections was 90 mg. Subsequent doses could be titrated (60, 90, 120 mg) according to symptom response. Seventy-one patients were treated. Flushing decreased from a mean of 3.0 at baseline to 2.3 on day 1, and 2.0 on day 2, with a daily mean of 2.1 for the first week post-treatment (p < 0.05). Diarrhea decreased from a mean of 5.0 at baseline to 4.3 on day 1 (p < 0.05), and 4.5 on day 2, with a daily mean of 4.4 for the first week post-treatment (p < 0.001). Symptom frequency decreased further after the second and third injections, and reached a plateau after the fourth injection. By month 6, flushing and diarrhea had significantly decreased from baseline by a mean of 1.3 and 1.1 episodes/day, respectively (both p < or = 0.001); 65% of patients with flushing as the target symptom and 18% of diarrhea-target patients achieved > or =50% reduction from baseline. Median urinary 5-hydroxyindoleacetic acid and chromogranin A levels decreased by 24 and 38%, respectively. Treatment was well tolerated. 28-day PR lanreotide was effective in reducing the symptoms and biochemical markers associated with carcinoid syndrome.
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PMID:Rapid and sustained relief from the symptoms of carcinoid syndrome: results from an open 6-month study of the 28-day prolonged-release formulation of lanreotide. 1597 11

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

(Neuro-)endocrine tumours of the gastrointestinal tract are also called 'carcinoids'. (Neuro-)endocrine midgut tumours can be categorized according to their clinical behaviour. Most tumours are non-functioning. Functioning tumours are responsible for the carcinoid syndrome. The carcinoid syndrome is almost uniquely associated with midgut carcinoids. Symptoms of the carcinoid syndrome are caused by an excess of biogenic amines, peptides and other factors in the circulation. The typical symptoms of the carcinoid syndrome are diarrhoea, flushing, and carcinoid heart disease. Carcinoid heart disease involves the tricuspid and pulmonary valves and the endocardium. Serum chromogranin A and urinary excretion of 5-hydroxy-indoleacetic acid (5-HIAA) are biochemical markers. Carcinoid tumours express large numbers of high-affinity somatostatin receptors. These can bind the currently available octapeptide somatostatin analogues. In inoperable patients, biotherapy with somatostatin analogues and interferon-alpha is the treatment of choice. Somatostatin analogues and interferon-alpha significantly improve symptoms.
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PMID:Tumours of the midgut (jejunum, ileum and ascending colon, including carcinoid syndrome). 1625 95

Gastro-intestinal carcinoids are slow growing tumors arising from enterochromaffin or Kulchitsky cells. Their clinical presentation depends on what combination of bioactive substances is secreted. Midgut carcinoid can present with the carcinoid syndrome in the presence of liver metastases. Its most typical clinical manifestations include cutaneous flushing and diarrhea. A nonspecific biochemical tumor marker for carcinoid tumors is serum chromogranin A and a specific marker for the carcinoid syndrome is the increased urinary excretion of 5-hydroxy indole acetic acid (5-HIAA). Localizing studies in carcinoid tumors/syndrome are: transabdominal ultrasonography (US), endoscopy, endoscopic US, videocapsule endoscopy, computerized tomography, magnetic resonance imaging, selective abdominal angiography, 111In-pentetreotide scintigraphy (and intraoperative radionuclide probe), 123I (131I)-metaiodobenzylguanidine (MIBG) scintigraphy, bone scintigraphy and 11C-5-HT positron emission tomography (PET). Therapies for carcinoid tumors/syndrome are: surgery, somatostatin analogs, interferon-alpha, radiotherapy, liver dearterialization, liver (chemo, or radio)-embolization, alcohol sclerotherapy of liver metastases, radiofrequency ablation of liver metastases, cryosurgery of liver metastases, occasionally liver transplantation, radiotherapy-coupled somatostatin analogs, 131I-MIBG and occasionally chemotherapy.
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PMID:Carcinoid syndrome: diagnosis and medical management. 1644 70


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