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Neuroendocrine tumours

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https://www.readbyqxmd.com/read/28637487/the-nf1-somatic-mutational-landscape-in-sporadic-human-cancers
#1
REVIEW
Charlotte Philpott, Hannah Tovell, Ian M Frayling, David N Cooper, Meena Upadhyaya
BACKGROUND: Neurofibromatosis type 1 (NF1: Online Mendelian Inheritance in Man (OMIM) #162200) is an autosomal dominantly inherited tumour predisposition syndrome. Heritable constitutional mutations in the NF1 gene result in dysregulation of the RAS/MAPK pathway and are causative of NF1. The major known function of the NF1 gene product neurofibromin is to downregulate RAS. NF1 exhibits variable clinical expression and is characterized by benign cutaneous lesions including neurofibromas and café-au-lait macules, as well as a predisposition to various types of malignancy, such as breast cancer and leukaemia...
June 21, 2017: Human Genomics
https://www.readbyqxmd.com/read/28634106/biology-evolution-and-medical-importance-of-polyomaviruses-an-update
#2
REVIEW
Ugo Moens, Andi Krumbholz, Bernhard Ehlers, Roland Zell, Reimar Johne, Sébastien Calvignac-Spencer, Chris Lauber
The family Polyomaviridae encompasses non-enveloped viruses with a circular dsDNA genome that is typically approximately 5000bp in length. Originally isolated from mammals, polyomavirus sequences have now been detected in invertebrates, fish, amphibians, reptiles and birds, although it remains to be determined whether all these animals are genuine hosts. The genomes of all polyomaviruses encode at least two regulatory proteins (large and small tumour antigen) and two structural proteins (capsid proteins VP1 and VP2) whose functions have been defined...
June 17, 2017: Infection, Genetics and Evolution
https://www.readbyqxmd.com/read/28631210/carcinoid-heart-disease-a%C3%A2-guide-for-screening-and-timing-of-surgical-intervention
#3
REVIEW
E A Hart, T A Meijs, R C A Meijer, K M Dreijerink, M E Tesselaar, C A de Groot, G D Valk, S A J Chamuleau
The cardiac manifestations of a neuroendocrine tumour are referred to as carcinoid heart disease (CaHD) and are associated with a poor prognosis. Surgical intervention is the only proven therapeutic option and may prolong survival and quality of life. No consensus has been reached internationally with regard to screening for CaHD and the optimal timing for surgery. Although limited evidence is available on this matter, a trend towards early surgery and subsequent reduced mortality has been observed. In this review we provide an overview of the current understanding and propose a protocol to guide cardiologists in the screening for CaHD and the timing of referral to a specialised surgical centre...
June 19, 2017: Netherlands Heart Journal
https://www.readbyqxmd.com/read/28625631/cd52-cd22-cd26-eg5-and-igf-1r-expression-in-thymic-malignancies
#4
J Remon, N Abedallaa, E Taranchon-Clermont, V Bluthgen, C R Lindsay, B Besse, V Thomas de Montpréville
BACKGROUND: Thymic epithelial tumours are rare cancers for which new treatment options are required. Identification of putative predictive markers is important for developing clinical trials. We studied the expression of five putative predictive biomarkers, potentially actionable by approved experimental drugs. METHODS: CD52, CD22, CD26, EG5, and IGF-1R expression were investigated by immunohistochemistry in formalin-fixed surgical samples of thymic epithelial tumour patients...
June 2017: Lung Cancer: Journal of the International Association for the Study of Lung Cancer
https://www.readbyqxmd.com/read/28619285/5-fluorouracil-biomarkers-in-pancreatic-neuroendocrine-tumours
#5
EDITORIAL
Louis de Mestier, Jérôme Cros, Pascal Hammel
No abstract text is available yet for this article.
June 8, 2017: Pancreatology: Official Journal of the International Association of Pancreatology (IAP) ... [et Al.]
https://www.readbyqxmd.com/read/28614007/ectopic-cushing-s-syndrome-a-ten-year-experience-from-a-tertiary-care-centre-in-southern-india
#6
Samantha Sathyakumar, Thomas Vizhalil Paul, Hesargatta Shyamsunder Asha, Birla Roy Gnanamuthu, M J Paul, Deepak Thomas Abraham, Simon Rajaratnam, Nihal Thomas
OBJECTIVE: Ectopic ACTH secretion is a less common cause of Cushing's syndrome, and is seen in 5 to 10% of cases with endogenous hypercortisolemia. We hereby describe our experience of patients with ectopic ACTH syndrome, who have been managed over the past 10 years at a tertiary care centre in Southern India. METHODS: The inpatient and outpatient records of patients from 2006 to 2015 were retrospectively reviewed. The clinical features, clinical history, biochemical values, imaging features which included radiological findings and PET scans, management, details of follow-up and outcomes were documented...
June 14, 2017: Endocrine Practice
https://www.readbyqxmd.com/read/28611057/middle-ear-adenoma-with-uncommon-presentation-and-literature-review
#7
Lin Wang, Nengtai Ouyang, Hai Xia Guo, Qing-Yu Liu
Middle ear adenoma (MEA) is a rare benign tumour. Here we report a case of MEA in a 39-year-old man with hearing loss. Some significant histopathological features of MEA and neuroendocrine differentiation identified by immunohistochemistry are described. The tumour showed remarkable and rare degenerative histological characteristics. There were a few tumour cells which were covered by degenerated tissue. Consequently, following frozen biopsy, the tumour was misdiagnosed as chronic ear inflammation. No recurrence was seen in this patient after 45 months of follow-up; regular clinical and radiological follow-up is necessary since recurrence is possible...
June 13, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28600342/gastrointestinal-manifestations-of-neuroendocrine-tumours-their-investigation-and-management
#8
REVIEW
Vandana M Sagar, Sheldon C Cooper, Jill Johnson, Shishir Shetty, Tahir Shah
The incidence of neuroendocrine tumours (NETs) is on the rise in the UK. Patients with NETs need to be managed by a team of clinical specialties. There are a number of challenging gastrointestinal (GI) manifestations related to NETs that can occur in these patients, but a limited literature base exists to guide clinicians.Whilst life expectancy can be several years for patients with NETs, the GI symptoms can have a significant impact on their quality of life. It is therefore imperative to be familiar with the common GI manifestations associated with NETs, so symptoms can be appropriately managed with an overall aim of restoring the patient's quality of life...
June 9, 2017: Postgraduate Medical Journal
https://www.readbyqxmd.com/read/28597909/diagnostic-and-therapeutic-guidelines-for-gastro-entero-pancreatic-neuroendocrine-neoplasms-recommended-by-the-polish-network-of-neuroendocrine-tumours
#9
Beata Kos-Kudła, Jolanta Blicharz-Dorniak, Janusz Strzelczyk, Agata Bałdys-Waligórska, Tomasz Bednarczuk, Marek Bolanowski, Agnieszka Boratyn-Nowicka, Małgorzata Borowska, Andrzej Cichocki, Jarosław B Ćwikła, Massimo Falconi, Wanda Foltyn, Daria Handkiewicz-Junak, Alicja Hubalewska-Dydejczyk, Barbara Jarząb, Roman Junik, Dariusz Kajdaniuk, Grzegorz Kamiński, Agnieszka Kolasińska-Ćwikła, Aldona Kowalska, Robert Król, Leszek Królicki, Maciej Krzakowski, Jolanta Kunikowska, Katarzyna Kuśnierz, Paweł Lampe, Dariusz Lange, Anna Lewczuk-Myślicka, Andrzej Lewiński, Michał Lipiński, Magdalena Londzin-Olesik, Bogdan Marek, Anna Nasierowska-Guttmejer, Sergiusz Nawrocki, Ewa Nowakowska-Duława, Joanna Pilch-Kowalczyk, Violetta Rosiek, Marek Ruchała, Lucyna Siemińska, Anna Sowa-Staszczak, Teresa Starzyńska, Katarzyna Steinhof-Radwańska, Krzysztof Sworczak, Anhelli Syrenicz, Andrzej Szawłowski, Marek Szczepkowski, Ewa Wachuła, Wojciech Zajęcki, Anna Zemczak, Wojciech Zgliczyński, Krzysztof Zieniewicz
Progress in the diagnostics and therapy of gastro-entero-pancreatic (GEP) neuroendocrine neoplasms (NEN), the published results of new randomised clinical trials, and the new guidelines issued by the European Neuroendocrine Tumour Society (ENETS) have led the Polish Network of Neuroendocrine Tumours to update the 2013 guidelines regarding management of these neoplasms. We present the general recommendations for the management of NENs, developed by experts during the Third Round Table Conference - Diagnostics and therapy of gastro-entero-pancreatic neuroendocrine neoplasms: Polish recommendations in view of current European recommenda-tions, which took place in December 2016 in Żelechów near Warsaw...
2017: Endokrynologia Polska
https://www.readbyqxmd.com/read/28596302/carcinoid-heart-disease
#10
REVIEW
Saamir A Hassan, Jose Banchs, Cezar Iliescu, Arvind Dasari, Juan Lopez-Mattei, Syed Wamique Yusuf
Rare neuroendocrine tumours (NETs) that most commonly arise in the gastrointestinal tract can lead to carcinoid syndrome and carcinoid heart disease. Patients with carcinoid syndrome present with vasomotor changes, hypermotility of the gastrointestinal system, hypotension and bronchospasm. Medical therapy for carcinoid syndrome, typically with somatostatin analogues, can help control symptoms, inhibit tumour progression and prolong survival. Carcinoid heart disease occurs in more than 50% of these patients and is the initial presentation of carcinoid syndrome in up to 20% of patients...
June 8, 2017: Heart: Official Journal of the British Cardiac Society
https://www.readbyqxmd.com/read/28584167/severe-cushing-s-syndrome-due-to-small-cell-prostate-carcinoma-a-case-and-review-of-literature
#11
Marianne Elston, Veronica Crawford, Michael Swarbrick, Michael Dray, Michelle Head, John V Conaglen
Cushings syndrome (CS) due to ectopic adrenocorticotrophic hormone (ACTH) is associated with a variety of tumours most of which arise in the thorax or abdomen. Prostate carcinoma is a rare but important cause of rapidly progressive CS. To report a case of severe CS due to ACTH production from prostate neuroendocrine carcinoma and summarise previous published cases. A 71 year old man presented with profound hypokalaemia, oedema and new onset hypertension. He reported two weeks of weight gain, muscle weakness, labile mood and insomnia...
June 5, 2017: Endocrine Connections
https://www.readbyqxmd.com/read/28583792/role-of-palliative-resection-of-the-primary-tumour-in-advanced-pancreatic-and-small-intestinal-neuroendocrine-tumours-a-systematic-review-and-meta-analysis
#12
REVIEW
L M Almond, J Hodson, S J Ford, D Gourevitch, K J Roberts, T Shah, J Isaac, A Desai
PURPOSE: This study aimed to evaluate the impact on overall survival following palliative surgery to remove the primary lesion in unresectable metastatic small intestinal (SI-NET) and pancreatic neuroendocrine tumours (P-NET). METHODS: A systematic review of the literature and meta-analysis was performed. MEDLINE and Embase databases were searched to identify articles comparing patients undergoing palliative primary tumour resection without metastatectomy vs. no resection...
May 25, 2017: European Journal of Surgical Oncology
https://www.readbyqxmd.com/read/28579289/effects-of-anti-somatostatin-agents-on-glucose-metabolism
#13
REVIEW
B Vergès
The anti-somatostatin agents used to treat acromegaly, Cushing's disease and neuroendocrine tumours also have hyperglycaemic effects. This is particularly true for pasireotide. Hyperglycaemic events are seen in 57-73% of patients with Cushing's treated with pasireotide, with a need to initiate antidiabetic treatment in about 50% of these patients. In acromegaly, treatment with pasireotide induces hyperglycaemia in 29-61% of patients. Pasireotide-induced hyperglycemia occurs early, within the first 3 months of treatment, due to a decrease in insulin secretion secondary to a fall in secretion of GLP-1 and GIP, and potentially also due to a direct inhibitory effect of pasireotide on beta cells...
June 1, 2017: Diabetes & Metabolism
https://www.readbyqxmd.com/read/28567298/recurrent-insulinoma-in-a-10-year-old-boy-with-down-s-syndrome
#14
Noman Ahmad, Abdulmonem Mohammed Almutawa, Mohamed Ziyad Abubacker, Hossam Ahmed Elzeftawy, Osama Abdullah Bawazir
An insulinoma is a rare tumour with an incidence of four cases per million per year in adults. The incidence in children is not established. There is limited literature available in children with insulinoma, and only one case is reported in association with Down's syndrome in adults. Insulinoma diagnosis is frequently missed in adults as well as in children. The Whipple triad is the most striking feature although it has limited application in young children. Hypoglycaemia with elevated insulin, C-peptide and absent ketones is highly suggestive of hyperinsulinism...
2017: Endocrinology, Diabetes & Metabolism Case Reports
https://www.readbyqxmd.com/read/28567290/acth-secreting-medullary-thyroid-cancer-a-case-series
#15
Ayanthi A Wijewardene, Sarah J Glastras, Diana L Learoyd, Bruce G Robinson, Venessa H M Tsang
Medullary thyroid cancer (MTC) is a rare neuroendocrine tumour that originates from the parafollicular cells of the thyroid gland. The most common presentation of MTC is with a single nodule; however, by the time of diagnosis, most have spread to the surrounding cervical lymph nodes. Cushing's syndrome is a rare complication of MTC and is due to ectopic adrenocorticotrophic hormone (ACTH) secretion by tumour cells. Cushing's syndrome presents a challenging diagnostic and management issue in patients with MTC...
2017: Endocrinology, Diabetes & Metabolism Case Reports
https://www.readbyqxmd.com/read/28566493/urinary-amine-sampling-for-tumour-detection-revisiting-the-recommendations
#16
Jean-Benoit Corcuff, Laurence Chardon, Ines El Hajji Ridah, Julie Brossaud
Context Biogenic amines such as 5-hydroxy-indole acetic acid (5HIAA), the main metabolite of serotonin or metanephrines, catecholamines metabolites, are used as biomarkers of neuroendocrine tumours Objective To re-evaluate the recommendations for urinary sampling (preservatives, diet, drugs, …) as many of the reported analytical interferences supporting these recommendations are related to obsolete assays. Methods Bibliographic analysis of old and modern amine assays concerning preservation, extraction, assay and interferences...
May 31, 2017: Endocrine Connections
https://www.readbyqxmd.com/read/28564709/-primary-mixed-adeno-neuroendocrine-carcinoma-manec-of-the-urinary-bladder-a-rare-entity
#17
Markus Eckstein, Bernd Rasper, Jörg Busche, Ulf Stachetzki, D E Wistuba, Abbas Agaimy, Arndt Hartmann, Simone Bertz
A 67-year-old male patient underwent transurethral resection for a bladder tumour at the lower posterior wall found incidentally on imaging for an unrelated disease. The initial histomorphological examination suggested an adenocarcinoma with partial neuroendocrine differentiation. Imaging of the thorax and the abdomen, showed no evidence of another primary tumour. Due to the unusual diagnosis, the case was sent to our department of pathology for expert consultation, which confirmed the diagnosis of a primary mixed adeno-neuroendocrine carcinoma of the bladder (MANEC)...
May 31, 2017: Aktuelle Urologie
https://www.readbyqxmd.com/read/28552629/clinical-and-pathological-analysis-of-19-cases-of-medullary-thyroid-carcinoma-without-an-increase-in-calcitonin
#18
Qiufeng Zhou, Shuanglei Yue, Ye Cheng, Ju Jin, Haimiao Xu
BACKGROUND: Medullary thyroid carcinoma (MTC), defined as a malignant tumour with C-cell differentiation, is of neuroendocrine origin and is characterized by the synthesis and secretion of calcitonin (CT). MTC without CT secretion has been reported on rare occasions. The purpose of this study was to evaluate the histological, immunohistochemical, and molecular pathologic features as well as the clinical significance of non-secretory MTC (NCR-MTC). METHODS: A retrospective analysis of patients with NCR-MTC was performed...
May 25, 2017: Experimental and Toxicologic Pathology: Official Journal of the Gesellschaft Für Toxikologische Pathologie
https://www.readbyqxmd.com/read/28548901/neuroendocrine-differentiation-in-basal-cell-carcinoma
#19
Yoldez Houcine, Ines Chelly, Alia Zehani, Linda Belhaj Kacem, Haifa Azzouz, Wafa Rekik, Hend C, Slim Haouet, Nidhameddine Kchir
Basal cell carcinoma (BCC) is the prototypical basaloid tumor of the skin. It may show various patterns simulating other cutaneous tumors due to its pleomorphism. It may have an unusal pattern of differentiation such as squamous, sebaceous, apocrine, eccrine, pilar, and endocrine differentiation. In order to establish the relative frequency of neuroendocrine differentiation in BCC, we performed a retrospective study of 33 consecutive BCCs using conventional immunohistochemistry with two neuroendocrine antibodies: Chromogranine A and synaptophysine...
May 26, 2017: Journal of Immunoassay & Immunochemistry
https://www.readbyqxmd.com/read/28547177/guideline-for-pet-ct-imaging-of-neuroendocrine-neoplasms-with-68-ga-dota-conjugated-somatostatin-receptor-targeting-peptides-and-18-f-dopa
#20
Murat Fani Bozkurt, Irene Virgolini, Sona Balogova, Mohsen Beheshti, Domenico Rubello, Clemens Decristoforo, Valentina Ambrosini, Andreas Kjaer, Roberto Delgado-Bolton, Jolanta Kunikowska, Wim J G Oyen, Arturo Chiti, Francesco Giammarile, Stefano Fanti
PURPOSE & METHODS: Neuroendocrine neoplasms are a heterogenous group of tumours, for which nuclear medicine plays an important role in the diagnostic work-up as well as in the targeted therapeutic options. This guideline is aimed to assist nuclear medicine physicians in recommending, performing, reporting and interpreting the results of somatostatin receptor (SSTR) PET/CT imaging using (68)Ga-DOTA-conjugated peptides, as well as (18)F-DOPA imaging for various neuroendocrine neoplasms...
May 25, 2017: European Journal of Nuclear Medicine and Molecular Imaging
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