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Neuroendocrine tumours

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https://www.readbyqxmd.com/read/29137302/functional-and-non-functional-pancreatic-neuroendocrine-tumours-enets-or-ajcc-tnm-staging-system
#1
Min Yang, Neng-Wen Ke, Yi Zhang, Chun-Lu Tan, Bo-Le Tian, Xu-Bao Liu, Wei Huang, Quentin Nunes, Robert Sutton
Background: There are currently 2 Tumour-Node-Metastasis (TNM) staging systems for pancreatic neuroendocrine tumours (p-NETs) - European Neuroendocrine Tumour Society (ENETS) and American Joint Committee on Cancer (AJCC). P-NETs being heterogeneous, we investigated the prognostic value of the 2 systems in p-NETs, as a whole, and more interestingly in functional and non-functional sub-groups separately, with a view to ascertaining any potential clinical benefits of using one system over the other...
October 10, 2017: Oncotarget
https://www.readbyqxmd.com/read/29134352/accuracy-of-apparent-diffusion-coefficient-in-differentiating-pancreatic-neuroendocrine-tumour-from-intrapancreatic-accessory-spleen
#2
Ankur Pandey, Pallavi Pandey, Mounes Aliyari Ghasabeh, Farnaz Najmi Varzaneh, Pegah Khoshpouri, Nannan Shao, Manijeh Zargham Pour, Daniel Fadaei Fouladi, Ralph H Hruban, Anne Marie O'Broin-Lennon, Ihab R Kamel
OBJECTIVES: To evaluate and compare the accuracy of absolute apparent diffusion coefficient (ADC) and normalised ADC (lesion-to-spleen ADC ratio) in differentiating pancreatic neuroendocrine tumour (NET) from intrapancreatic accessory spleen (IPAS). METHODS: Study included 62 patients with the diagnosis of pancreatic NET (n=51) or IPAS (n=11). Two independent reviewers measured ADC on all lesions and spleen. Receiver operating characteristics (ROC) analysis to differentiate NET from IPAS was performed and compared for absolute and normalised ADC...
November 13, 2017: European Radiology
https://www.readbyqxmd.com/read/29132721/endocrine-pathology-past-present-and-future
#3
REVIEW
Sylvia L Asa, Ozgur Mete
Endocrine pathology is the subspecialty of diagnostic pathology which deals with the diagnosis and characterisation of neoplastic and non-neoplastic diseases of the endocrine system. This relatively young subspecialty was initially focused mainly on thyroid and parathyroid pathology, with some participants also involved in studies of the pituitary, the endocrine pancreas, and the adrenal glands. However, the endocrine system involves much more than these traditional endocrine organs and the discipline has grown to encompass lesions of the dispersed neuroendocrine cells, including neuroendocrine tumours (NETs) of the lungs, gastrointestinal tract, thymus, breast and prostate, as well as paraganglia throughout the body, not just in the adrenals...
November 10, 2017: Pathology
https://www.readbyqxmd.com/read/29126671/treatment-challenges-in-and-outside-a-specialist-network-setting-pancreatic-neuroendocrine-tumours
#4
Panagis M Lykoudis, Stefano Partelli, Francesca Muffatti, Martyn Caplin, Massimo Falconi, Giuseppe K Fusai
Pancreatic Neuroendocrine Neoplasms comprise a group of rare tumours with special biology, an often indolent behaviour and particular diagnostic and therapeutic requirements. The specialized biochemical tests and radiological investigations, the complexity of surgical options and the variety of medical treatments that require individual tailoring, mandate a multidisciplinary approach that can be optimally achieved through an organized network. The present study describes currents concepts in the management of these tumours as well as an insight into the challenges of delivering the pathway in and outside a Network...
October 16, 2017: European Journal of Surgical Oncology
https://www.readbyqxmd.com/read/29124493/genotype-phenotype-correlation-in-asian-indian-von-hippel-lindau-vhl-syndrome-patients-with-pheochromocytoma-paraganglioma
#5
Nilesh Lomte, Sanjeet Kumar, Vijaya Sarathi, Reshma Pandit, Manjunath Goroshi, Swati Jadhav, Anurag R Lila, Tushar Bandgar, Nalini S Shah
The data in genotype-phenotype correlation in Indian von Hippel-Lindau (VHL) patients is limited. We have retrospectively studied 31 genetically proven VHL patients with pheochromocytoma/paraganglioma (PCC/PGL) from families and have reviewed the World literature on PCC/PGL in patients with large VHL deletions. Three patients had large deletions and 28 patients had other mutations [missense mutations in 25, 3 bp deletion in 2 and single bp duplication in one]. Unilateral PCC were significantly more common in patients with large VHL deletions whereas multiple PCC (bilateral PCC or PCC + sympathetic PGL) were significantly more common in those with other mutations...
November 9, 2017: Familial Cancer
https://www.readbyqxmd.com/read/29107992/software-assisted-dosimetry-in-peptide-receptor-radionuclide-therapy-with-177lutetium-dotatate-for-various-imaging-scenarios
#6
Dennis Kupitz, Christoph Wetz, Heiko Wissel, Florian Wedel, Ivayla Apostolova, Thekla Wallbaum, Jens Ricke, Holger Amthauer, Oliver S Grosser
In peptide receptor radionuclide therapy (PRRT) of patients with neuroendocrine neoplasias (NENs), intratherapeutic dosimetry is mandatory for organs at risk (e.g. kidneys) and tumours. We evaluated commercial dosimetry software (Dosimetry Toolkit) using varying imaging scenarios, based on planar and/or tomographic data, regarding the differences in calculated organ/tumour doses and the use for clinical routines. A total of 16 consecutive patients with NENs treated by PRRT with 177Lu-DOTATATE were retrospectively analysed...
2017: PloS One
https://www.readbyqxmd.com/read/29104610/functional-pancreatic-neuroendocrine-tumour-causing-cushing-s-syndrome-the-effect-of-chemotherapy-on-clinical-symptoms
#7
Paulo Henrique do Amor Divino, Katia Regina Marchetti, Madson Q Almeida, Rachel P Riechelmann
Background: Neuroendocrine tumours (NETs) are a heterogeneous group of diseases that can originate from any part of the gastrointestinal tract, bronchi, thyroid and pancreas. These tumours may be functioning or not depending on their ability to produce active substances, such as adrenocorticotrophic hormone (ACTH). ACTH-producing pancreatic neuroendocrine tumours are rare, with limited data about effective antitumor therapies. Case Report: A 58-year-old man with a history of type-2 diabetes mellitus and arterial hypertension was diagnosed with Cushing's syndrome (CS) secondary to an ACTH ectopic production from a well-differentiated neuroendocrine tumour of the pancreas metastatic to the liver...
2017: Ecancermedicalscience
https://www.readbyqxmd.com/read/29100361/glyoxalase-1-copy-number-variation-in-patients-with-well-differentiated-gastro-entero-pancreatic-neuroendocrine-tumours-gep-net
#8
Mingzhan Xue, Alaa Shafie, Talha Qaiser, Nasir M Rajpoot, Gregory Kaltsas, Sean James, Kishore Gopalakrishnan, Adrian Fisk, Georgios K Dimitriadis, Dimitris K Grammatopoulos, Naila Rabbani, Paul J Thornalley, Martin O Weickert
Background: The glyoxalase-1 gene (GLO1) is a hotspot for copy-number variation (CNV) in human genomes. Increased GLO1 copy-number is associated with multidrug resistance in tumour chemotherapy, but prevalence of GLO1 CNV in gastro-entero-pancreatic neuroendocrine tumours (GEP-NET) is unknown. Methods: GLO1 copy-number variation was measured in 39 patients with GEP-NET (midgut NET, n = 25; pancreatic NET, n = 14) after curative or debulking surgical treatment. Primary tumour tissue, surrounding healthy tissue and, where applicable, additional metastatic tumour tissue were analysed, using real time qPCR...
September 29, 2017: Oncotarget
https://www.readbyqxmd.com/read/29095917/comparison-of-the-human-gastric-microbiota-in-hypochlorhydric-states-arising-as-a-result-of-helicobacter-pylori-induced-atrophic-gastritis-autoimmune-atrophic-gastritis-and-proton-pump-inhibitor-use
#9
COMPARATIVE STUDY
Bryony N Parsons, Umer Z Ijaz, Rosalinda D'Amore, Michael D Burkitt, Richard Eccles, Luca Lenzi, Carrie A Duckworth, Andrew R Moore, Laszlo Tiszlavicz, Andrea Varro, Neil Hall, D Mark Pritchard
Several conditions associated with reduced gastric acid secretion confer an altered risk of developing a gastric malignancy. Helicobacter pylori-induced atrophic gastritis predisposes to gastric adenocarcinoma, autoimmune atrophic gastritis is a precursor of type I gastric neuroendocrine tumours, whereas proton pump inhibitor (PPI) use does not affect stomach cancer risk. We hypothesised that each of these conditions was associated with specific alterations in the gastric microbiota and that this influenced subsequent tumour risk...
November 2017: PLoS Pathogens
https://www.readbyqxmd.com/read/29092962/multiple-endocrine-neoplasia-type-1-presenting-with-refractory-seizures
#10
Banshi Lal Kumawat, Chandramohan Sharma, Mohit Janakkumar Shah, Maulik Panchal
We report a case of 29-year-old woman referred to us for management of refractory epilepsy. Under observation, she was detected to have recurrent hypoglycaemia during the episodes of seizures. On investigation, she was found to have hyperinsulinemic hypoglycaemia. Her triple-phase CT scan of abdomen showed neuroendocrine tumour of pancreatic head, with bilateral renal calculi. Screening of other endocrine glands revealed pituitary microadenoma and parathyroid adenoma on imaging, which was also supported by biochemical and hormonal profile...
November 1, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/29092957/molecular-characterisation-of-metastatic-pancreatic-neuroendocrine-tumours-pnets-using-whole-genome-and-transcriptome-sequencing
#11
Hui-Li Wong, Kevin C Yang, Yaoqing Shen, Eric Y Zhao, Jonathan M Loree, Hagen F Kennecke, Steve E Kalloger, Joanna M Karasinska, Howard J Lim, Andrew J Mungall, Xiaolan Feng, Janine M Davies, Kasmintan Schrader, Chen Zhou, Aly Karsan, Steven Jm Jones, Janessa Laskin, Marco A Marra, David F Schaeffer, Sharon M Gorski, Daniel J Renouf
Pancreatic neuroendocrine tumours (PNETs) are a genomically and clinically heterogeneous group of pancreatic neoplasms often diagnosed with distant metastases. Recurrent somatic mutations, chromosomal aberrations and gene expression signatures in PNETs have been described, but the clinical significance of these molecular changes is still poorly understood, and the clinical outcomes of PNET patients remain highly variable. To help identify the molecular factors that contribute to PNET progression and metastasis, and as part of an ongoing clinical trial at the BC Cancer Agency (clinicaltrials...
November 1, 2017: Cold Spring Harbor Molecular Case Studies
https://www.readbyqxmd.com/read/29077690/small-cell-lung-cancer-what-we-know-what-we-need-to-know-and-the-path-forward
#12
REVIEW
Adi F Gazdar, Paul A Bunn, John D Minna
Small-cell lung cancer (SCLC) is a deadly tumour accounting for approximately 15% of lung cancers and is pathologically, molecularly, biologically and clinically very different from other lung cancers. While the majority of tumours express a neuroendocrine programme (integrating neural and endocrine properties), an important subset of tumours have low or absent expression of this programme. The probable initiating molecular events are inactivation of TP53 and RB1, as well as frequent disruption of several signalling networks, including Notch signalling...
October 27, 2017: Nature Reviews. Cancer
https://www.readbyqxmd.com/read/29074101/towards-personalised-medicine-in-lung-and-thymus-neuroendocrine-tumours
#13
Giuseppe Pelosi, Judith Cave, Christian H Ottensmeier
No abstract text is available yet for this article.
October 23, 2017: Lancet Oncology
https://www.readbyqxmd.com/read/29072302/merkel-cell-carcinoma
#14
REVIEW
Jürgen C Becker, Andreas Stang, James A DeCaprio, Lorenzo Cerroni, Celeste Lebbé, Michael Veness, Paul Nghiem
Merkel cell carcinoma (MCC) is a rare but highly aggressive skin cancer with neuroendocrine features. MCC pathogenesis is associated with either the presence of Merkel cell polyomavirus or chronic exposure to ultraviolet light (UV), which can cause a characteristic pattern of multiple DNA mutations. Notably, in the Northern hemisphere, the majority of MCC cases are of viral aetiology; by contrast, in areas with high UV exposure, UV-mediated carcinogenesis is predominant. The two aetiologies share similar clinical, histopathological and prognostic characteristics...
October 26, 2017: Nature Reviews. Disease Primers
https://www.readbyqxmd.com/read/29066640/neuroendocrine-tumour-in-pancreatic-dorsal-agenesis-a-rare-association
#15
Pedro Rodrigues, Rui Caetano Oliveira, Carlos Miguel Oliveira, Maria Augusta Cipriano
Pancreatic dorsal agenesis (PDA) is a rare congenital anomaly, usually asymptomatic, that can present with abdominal pain, pancreatitis, diabetes mellitus and jaundice. Pancreatic tumours in PDA background are extremely rare, and when they occur are mainly pancreatic ductal adenocarcinoma. We present a case of a 48-year-old female patient with incidental detection of a 26×20 mm nodular lesion of the cephalic pancreas on ultrasound. Surgery was performed and gross examination revealed PDA with a tumour developed around the Wirsung duct...
October 23, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/29066637/transcatheter-valve-implantation-to-inferior-vena-cava-to-control-carcinoid-symptoms
#16
Vandana M Sagar, Richard P Steeds, Sagar N Doshi, Tahir Shah
Severe carcinoid syndrome and carcinoid heart disease in neuroendocrine tumours can have a significant impact on a patient's quality of life and are a major cause of morbidity and mortality. We present a novel approach to managing a patient with medically uncontrollable carcinoid syndrome. Inferior and superior vena cava placement of transcatheter heart valves has been used to treat patients with right heart failure due to severe tricuspid and pulmonary regurgitation. However, this procedure has not been attempted to specifically reduce hormone secretion, primarily from the liver, in order to control carcinoid syndrome symptoms...
October 23, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/29066504/patient-quality-of-life-and-prognosis-in-men2
#17
Joanna Grey, Kym Winter
Multiple endocrine neoplasia type 2 (MEN2) refers to the autosomal dominant neuroendocrine tumour syndromes, MEN type 2A (MEN2A) and MEN type 2B (MEN2B). They are typified by the development of medullary thyroid carcinoma (MTC), phaeochromocytoma and parathyroid hyperplasia in MEN2A, and MTC, phaeochromocytomas, ganglioneuromatosis and skeletal abnormalities in MEN2B. The aggressiveness of MTC is variable according to genotype, and although it is still the major cause of mortality in both conditions, prognosis has improved dramatically in those diagnosed and treated at a young age thanks to predictive genetic testing...
October 24, 2017: Endocrine-related Cancer
https://www.readbyqxmd.com/read/29066490/novel-germline-p-gly42val-men1-mutation-in-a-family-with-multiple-endocrine-neoplasia-type-1-excellent-response-of-prolactinoma-to-cabergoline
#18
Viktoria F Koehler, Kerstin Jungheim, Ute Groß, Donato Iacovazzo, Alexander Mann, Márta Korbonits
We report on a 27-year-old male patient presenting with renal colic secondary to hyperparathyroidism. Further investigations confirmed a diagnosis of type 1 multiple endocrine neoplasia and revealed a 2.0 cm pancreatic neuroendocrine tumour as well as a pituitary macroadenoma with significantly elevated prolactin levels. The patient underwent three-gland parathyroidectomy, a left pancreatectomy, and received dopamine agonist treatment. Genetic testing revealed a novel germline heterozygote missense mutation in the MEN1 gene (p...
September 2017: Annals of Clinical and Laboratory Science
https://www.readbyqxmd.com/read/29064650/the-demographic-features-clinical-outcomes-prognosis-and-treatment-options-for-patients-with-sarcomatoid-carcinoma-of-the-urinary-bladder-a-single-centre-experience
#19
Simon Paul Robinson, Assad Farooq, Marc Laniado, Hanif Motiwala
INTRODUCTION: Carcinosarcoma of the bladder is a very rare neoplasm. The pathogenesis of carcinosarcomas is not clearly understood and remains a subject of debate. Whilst there is some research conceptualizing the histopathological findings of bladder carcinosarcomas, the demographic features, clinical outcomes, prognosis and treatment options remain unclear. MATERIALS AND METHODS: We analyzed 12 consecutive cases of patients with sarcoma-toid bladder cancer who were treated surgically at a single Urology Department be-tween 1999 and 2015...
October 24, 2017: International Braz J Urol: Official Journal of the Brazilian Society of Urology
https://www.readbyqxmd.com/read/29051783/a-case-of-pulmonary-carcinoid-in-pregnancy-and-review-of-carcinoid-tumours-in-pregnancy
#20
D Kevat, M Chen, D Wyld, N Fagermo, K Lust
Carcinoid tumours are rare slow growing tumours which arise from primitive neuroendocrine cells. The effect of the pregnant state on carcinoid tumours and vice versa remains unclear, as does the optimal management of carcinoid tumours during the pregnancy including labour. We report the rare case of a 36 year old primigravida woman with large bilateral pulmonary carcinoid tumours. The patient's disease was minimally symptomatic with no clinical suspicion of carcinoid syndrome. Under close observation, the pregnancy progressed well and the patient proceeded to a spontaneous vaginal delivery of a healthy child...
September 2017: Obstetric Medicine
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