Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UNIPROT:P61278 (somatostatin)
 22,083 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

In this case report the typical echocardiographic features of carcinoid heart disease are presented. Newer treatments such as the use of a somatostatin analogue, interferon and hepatic de-arterialisation have improved the prognosis in patients with carcinoid syndrome. Nevertheless this syndrome portends a poor prognosis in patients with cardiac involvement. Cardiac lesions are mainly located in the right side of the heart. Regurgitation and stenosis of the tricuspid and pulmonary valve, leading to right heart failure, are the most common cardiac manifestations of the disease. Elevated levels of serotonin are probably responsible for the development of these cardiac lesions. Despite treatment resulting in significant reductions of urinary levels of 5-HIAA, regression of the cardiac manifestations in carcinoid syndrome has not been observed. Two-dimensional and Doppler echocardiography are the main tools to establish the diagnosis and severity of carcinoid heart disease. Cardiac surgery for carcinoid heart disease might improve symptoms and longevity, but the scarce data report on early mortality of over 35%.
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PMID:Echocardiographic diagnosis in carcinoid heart disease. 1216 98

Midgut carcinoids developing in the small intestine represent the most common cause of the carcinoid syndrome, with severe symptoms of flush, diarrhoea, and fibrotic valvular heart disease. The tumours may be histologically identified with specific chromogranin A or synaptophysine immunostainings, and by serotonin reactivity, which supports a midgut origin. Urinary 5-HIAA excretion and serum chromogranin A measurements are used as biochemical tumour markers for clinical diagnosis, and as important monitors of treatment effects and prognostic predictors. The midgut carcinoids have typically slow proliferation and extended disease course, and surgical treatment has become increasingly important for their management. Surgery should aim to remove primary tumours and mesenteric metastases, which may cause long-term abdominal complications, by typical fibrotic intestinal entrapment and small bowel ischaemia due to encasement of mesenteric vessels. Attempts should also be made to surgically remove or ablate liver metastases, since this may significantly contribute to palliation of the carcinoid syndrome. In patients with this syndrome surgery is combined with continuous biotherapy with long-acting somatostatin analogues and interferon, which may alleviate symptoms and cause stable disease with slow progression. Favourable survival and life-quality can be expected with this treatment also in patients with advanced midgut carcinoids.
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PMID:Management of midgut carcinoids. 1571 73

Patients with carcinoid tumours frequently present with metastatic disease. There are only a few therapeutic options for these patients, and the main goal of palliative treatment is to reduce symptoms and thus to improve quality of life. Current therapy includes surgical resection, hepatic artery embolisation, chemotherapy and somatostatin analogue treatment; however, all these options have limitations. It seems probable that therapeutic modalities based on radiopharmaceuticals may provide better therapy, not only in relation to symptom reduction but may also improve patient survival. In this case report we present a 46-year-old woman with a symptomatic carcinoid, who at the time of diagnosis had liver and abdominal lymph node metastases, the primary tumour being located in the terminal ileum. (111)In-pentetreotide scanning was negative, whereas (123)I-MIBG scanning showed high avidity in the tumour tissue. After right hemicolectomy, two courses of (131)I-MIBG treatment were given (12.95 GBq and 12 GBq, respectively). After the second dose of (131)I-MIBG temporary pancytopenia was present. Octreotide therapy was given empirically only for a short time and was stopped because of drug intolerance. The patient underwent tricuspid and pulmonary valve replacement because of her carcinoid heart disease, followed by two courses of embolisation of liver metastases. While (131)I-MIBG therapy reduced the patient's symptoms of flushing and diarrhoea, there has not yet been any effect on tumour response or 5-HIAA production. This case illustrates the multimodality and multidisciplinary approach to such patients.
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PMID:Multimodality palliative treatment of (111)In-pentetreotide negative/(123)I-MIBG positive metastatic carcinoid - a case report. 1877 5

Carcinoid tumors account for less than 1% of all malignancies and the majority arises in the gastrointestinal system. These tumors are slow-growing compared with adenocarcinomas and they differ from the other neuroendocrine malignancies by their protean clinical presentation. Carcinoid tumors were previously considered indolent, but they can manifest malignant characteristics with metastatic spread which often results in a poor prognosis. Although there have been advances in diagnostic and treatment modalities, carcinoid tumors are still frequently diagnosed late, often when the tumor has metastasized and patients have developed carcinoid syndrome. Diagnosis, prognosis and treatment options are based on biochemical markers and imaging investigations. High concentration of urinary 5-HIAA, elevated plasma serotonin and chromogranin A levels help to establish the initial diagnosis of carcinoid tumors. In addition to the computed tomography and magnetic resonance imaging, molecular imaging modalities such as OctreoScan, metaiodobenzylguanidine (MIBG) imaging and more recently PET imaging are used in detecting the primary malignancy and metastatic involvement. Surgery is the mainstay of treatment of non-metastatic carcinoid tumors. Cytotoxic chemotherapy has limited role because of the chemoresistant nature of these tumors. Because carcinoid tumors express somatostatin receptors, somatostatin analogues, which inhibit release of serotonin and other neuroendocrine peptides, are often used, but their use is limited to symptom control. Treatment using high doses of radionuclides, such as radiolabeled somatostatin analogues and MIBG, is a more recent option, which offers a definite advantage in management. In this article, we review the current state of the art in the diagnosis and treatment of carcinoid tumors as well as the role of MIBG in their diagnosis and management.
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PMID:Radioiodinated metaiodobenzylguanidine in the diagnosis and therapy of carcinoid tumors. 1908 97

Neuroendocrine tumor (NET) of the appendix is the most common gastrointestinal epithelial tumor in children. The utility of serum markers or the indication for hemicolectomy has not been established in children. In 45 children diagnosed with appendiceal NET, 89% NETs were incidentally found following appendectomy performed for suspected acute appendicitis. The median age was 12 years, and 56% patients were female. Postoperative somatostatin scan (n=5), serum chromogranin A (n=4), and urine 5-HIAA (n=9) were all within normal limits. Pathology slides of 35 patients showed mesoappendiceal invasion in 29% patients, and vascular invasion in 6% patients. Seven patients (16%) underwent hemicolectomy for invasion of mesoappendix (n=5), tumor near the resection margin (n=1), and tumor size 1.5 cm with vascular invasion (n=1). Only 2 hemicolectomy specimens showed disease: one in the appendiceal stump and the other as a micrometastasis in a mesenteric lymph node. There were no recurrences and all patients were alive and without evidence of disease at last follow-up. Pediatric appendiceal NET tends to have a benign clinical course with excellent prognosis. In the absence of carcinoid syndrome, postoperative scans and serum biomarkers do not seem to be useful. With completely resected tumors, the indication for hemicolectomy is unclear.
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PMID:Neuroendocrine Tumor of the Appendix in Children. 2730 28

Thoracic NETs [bronchopulmonary NETs (BPNETs) and thymic NETs (TNET)] share a common anatomic primary location, likely a common cell of origin, the "Kulchitsky cell" and presumably, a common etiopathogenesis. Although they are similarly grouped into well-differentiated [typical carcinoids (TC) and atypical carcinoids (AC)] and poorly differentiated neoplasms and both express somatostatin receptors, they exhibit a wide variation in clinical behavior. TNETs are more aggressive, are frequently metastatic, and have a lower 5-year survival rate (~50% vs. ~80%) than BPNETs. They are typically symptomatic, most often secreting ACTH (40% of tumors) but both tumor groups share secretion of common biomarkers including chromogranin A and 5-HIAA. Consistently effective and accurate circulating biomarkers are, however, currently unavailable. Surgery is the primary therapeutic tool for both BPNET and TNETs but there remains little consensus about later interventions e.g., targeted therapy, or how these can be monitored. Genetic analyses have identified different topographies (e.g., significant alterations in chromatin and epigenetic remodeling in BPNETs versus frequent chromosomal abnormalities in TNETs) but there is an absence of clinically actionable mutations in both tumor groups. Liquid biopsies, tools that can measure neoplastic signatures in peripheral blood, can potentially be leveraged to detect disease early i.e., recurrence, predict tumors that may respond to specific therapies and serve as real-time monitors for treatment responses. Recent studies have identified that mRNA transcript analysis in blood effectively identifies both BPNET and TNETs. The clinical utility of this gene expression assay includes use as a diagnostic, confirmation of completeness of surgical resection and use as a molecular management tool to monitor efficacy of PRRT and other therapeutic strategies.
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PMID:Molecular strategies in the management of bronchopulmonary and thymic neuroendocrine neoplasms. 2920 49

Rationale.Pancreatic neuroendocrine tumors (NETs) are rare neoplasms that develop from the endocrine tissues of the pancreas. They have a better overall prognosis than pancreatic adenocarcinoma. However, all commonly used classification systems reflect a separation between more indolent, well-differentiated tumors and far more aggressive poorly differentiated types that behave clinically more like small-cell carcinoma of the lung. Objective.To present the case of a 62-year-old man with an aggressive pancreatic NET, with liver, splenic and bone metastases who underwent multidisciplinary treatment including several lines of chemotherapy, somatostatin analogs and radiotherapy. Methods and Results.The patient is a smoker and an occasional drinker, known with type two diabetes mellitus (DM), receiving insulin therapy. He was diagnosed by contrast-enhanced computed tomography (CT) in January 2015 with a locally invasive pancreatic body mass, intraabdominal adenopathies and liver nodules, suggestive of metastases. Histopathological diagnosis was obtained through liver biopsy: neuroendocrine tumor with a 10-15% Ki67 proliferation index. Palliative chemotherapy with oxaliplatin and capecitabine was started in March 2015. In June 2015, Sandostatin LAR was added. In March 2016, he had progressive disease. Subsequently, in September 2016, bone metastasis was found within the T10 vertebra. He underwent radiotherapy for multiple bone metastases in February 2017. Progressive disease was again found during a CT examination in May 2017. His performance status has gradually worsened since then and he died in July 2017. Discussion.As a group, well-differentiated gastroenteropancreatic NETs are generally indolent malignancies with prolonged natural history. Intermediate-grade NETs have a slightly worse prognosis than low-grade tumors. Abbreviations: NETs - neuroendocrine tumors, NEC - neuroendocrine carcinoma, CT - computed tomography, MRI - magnetic resonance imaging, DM - diabetes mellitus, WHO - World Health Organisation, HCV - hepatitis C virus, CEA - carcinoembryonic antigen, AFP - alpha-fetoprotein, 5-HIAA - 5-Hydroxyindoleacetic acid, IHC - immunohistochemistry, EUS - endoscopic ultrasonography, EUS FNA - endoscopic ultrasonography with fine needle aspiration, CgA - chromogranin A, PRRT - peptide receptor radioligand therapy.
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PMID:Metastatic neuroendocrine pancreatic tumor - Case report. 2969 66


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