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Pancreatic neuroendocrine tumour

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https://www.readbyqxmd.com/read/29449689/current-and-emerging-therapies-for-pnets-in-patients-with-or-without-men1
#1
REVIEW
Morten Frost, Kate E Lines, Rajesh V Thakker
Pancreatic neuroendocrine tumours (PNETs) might occur as a non-familial isolated endocrinopathy or as part of a complex hereditary syndrome, such as multiple endocrine neoplasia type 1 (MEN1). MEN1 is an autosomal dominant disorder characterized by the combined occurrence of PNETs with tumours of the parathyroids and anterior pituitary. Treatments for primary PNETs include surgery. Treatments for non-resectable PNETs and metastases include biotherapy (for example, somatostatin analogues, inhibitors of receptors and monoclonal antibodies), chemotherapy and radiological therapy...
February 16, 2018: Nature Reviews. Endocrinology
https://www.readbyqxmd.com/read/29444910/the-neuroendocrine-phenotype-genomic-profile-and-therapeutic-sensitivity-of-gepnet-cell-lines
#2
Tobias Hofving, Yvonne Arvidsson, Bilal Almobarak, Linda Inge, Roswitha Pfragner, Marta Persson, Göran Stenman, Erik Kristiansson, Viktor Johanson, Ola Nilsson
Experimental models of neuroendocrine tumour disease are scarce, and no comprehensive characterisation of existing gastroenteropancreatic neuroendocrine tumour (GEPNET) cell lines has been reported. In this study, we aimed to define the molecular characteristics and therapeutic sensitivity of these cell lines. We therefore performed immunophenotyping, copy number profiling, whole-exome sequencing and a large-scale inhibitor screening of seven GEPNET cell lines. Four cell lines, GOT1, P-STS, BON-1 and QGP-1, displayed a neuroendocrine phenotype while three others, KRJ-I, L-STS and H-STS, did not...
March 2018: Endocrine-related Cancer
https://www.readbyqxmd.com/read/29433805/proactive-multi-modality-treatment-of-pancreatic-neuroendocrine-tumours-pnets-potential-survival-benefits
#3
L Tanno, D Mayo, S Mills, A Takhar, J Cave, L Nolan, B Stedman, F X Sundram, M Abu Hilal, H Connor, N Pearce, T Armstrong
BACKGROUND/OBJECTIVES: Primary and metastatic pancreatic neuroendocrine tumours (PNET) can be treated with combination of surgery, locoregional and systemic therapy. Survival benefits from individual treatments have been well reported, however, the combined outcome from multimodal treatments are not well described in the literature. We report outcomes in a cohort of PNET patients treated with proactive, multimodality therapy. METHODS: 106 patients were identified from a single tertiary referral centre prospective database...
December 14, 2017: Pancreatology: Official Journal of the International Association of Pancreatology (IAP) ... [et Al.]
https://www.readbyqxmd.com/read/29399966/morphologically-invisible-proinsulin-secreting-adenoma-detected-by-ga-68-exendin-4-glp-1-receptor-positron-emission-tomography-ct
#4
Christoph Werner, Amelie Lupp, Robert Drescher, Martin Freesmeyer, Masoud Mireskandari, Christian Stoykow, Astrid Bauschke, Ulrich A Müller
This case report of a young man suffering from recurring hypoglycaemia illustrates a rare condition of a neuroendocrine tumour, predominantly secreting proinsulin and invisible to conventional imaging approaches. Only a GLP-1 receptor PET/CT using Exendin-4 visualized the pancreatic lesion and enabled curative therapy, confirming the diagnostic value of this tracer for detection of neuroendocrine tumours. As only few publications on this topic are available, an overview of the available data is also given. The known cut-off value of 60% for proinsulin level indicating malignancy is critically discussed...
February 5, 2018: Journal of Medical Imaging and Radiation Oncology
https://www.readbyqxmd.com/read/29398971/incidental-detection-of-asymptomatic-brain-metastases-on-18f-fluoride-positron-emission-tomography-computed-tomography-and-68ga-dotanoc-positron-emission-tomography-computed-tomography-in-a-patient-with-concomitant-breast-carcinoma-and-a-pancreatic-neuroendocrine
#5
Ruth Brown, Phei Shan Chuah, Emmanouil Panagiotidis, Sobhan Vinjamuri
A 54-year-old female treated for locally advanced ductal breast carcinoma was also diagnosed with a pancreatic neuroendocrine tumour. A staging 68Ga DOTANOC positron emission tomography/computed tomography (PET/CT) demonstrated somatostatin receptor-positive foci within the brain parenchyma. A whole body 18F-fluoride PET/CT also demonstrated several foci of low-grade tracer uptake in the brain. Magnetic resonance imaging confirmed several cerebral and cerebellar metastases. This case highlights the need to be aware of each tumor's metastatic profile and the careful attention required for thoroughly evaluating imaging in the presence of multiple pathologies...
January 2018: World Journal of Nuclear Medicine
https://www.readbyqxmd.com/read/29367876/insulinoma-presenting-with-post-prandial-hypoglycaemia-following-fundoplication
#6
Sarah Y Qian, Matthew J L Hare, Alan Pham, Duncan J Topliss
Insulinomas are rare neuroendocrine tumours that classically present with fasting hypoglycaemia. This case report discusses an uncommon and challenging case of insulinoma soon after upper gastrointestinal surgery. A 63-year-old man presented with 6 months of post-prandial hypoglycaemia beginning after a laparoscopic revision of Toupet fundoplication. Hyperinsulinaemic hypoglycaemia was confirmed during a spontaneous episode and in a mixed-meal test. Localisation studies including magnetic resonance imaging (MRI), endoscopic ultrasound (EUS) and gallium dotatate positron emission tomography ( 68 Ga Dotatate PET) were consistent with a small insulinoma in the mid-body of the pancreas...
2018: Endocrinology, Diabetes & Metabolism Case Reports
https://www.readbyqxmd.com/read/29352378/can-histogram-analysis-of-mr-images-predict-aggressiveness-in-pancreatic-neuroendocrine-tumors
#7
Riccardo De Robertis, Bogdan Maris, Nicolò Cardobi, Paolo Tinazzi Martini, Stefano Gobbo, Paola Capelli, Silvia Ortolani, Sara Cingarlini, Salvatore Paiella, Luca Landoni, Giovanni Butturini, Paolo Regi, Aldo Scarpa, Giampaolo Tortora, Mirko D'Onofrio
OBJECTIVES: To evaluate MRI derived whole-tumour histogram analysis parameters in predicting pancreatic neuroendocrine neoplasm (panNEN) grade and aggressiveness. METHODS: Pre-operative MR of 42 consecutive patients with panNEN >1 cm were retrospectively analysed. T1-/T2-weighted images and ADC maps were analysed. Histogram-derived parameters were compared to histopathological features using the Mann-Whitney U test. Diagnostic accuracy was assessed by ROC-AUC analysis; sensitivity and specificity were assessed for each histogram parameter...
January 19, 2018: European Radiology
https://www.readbyqxmd.com/read/29321190/genomic-landscape-of-pancreatic-neuroendocrine-tumours-the-international-cancer-genome-consortium
#8
Andrea Mafficini, Aldo Scarpa
Neuroendocrine tumours (NETs) may arise throughout the body and are a highly heterogeneous, relatively rare class of neoplasms difficult to study also for the lack of disease models. Despite this, knowledge on their molecular alterations has expanded in the latest years, also building from genetic syndromes causing their onset. Pancreatic NETs (PanNETs) have been among the most studied, and research so far has outlined a series of recurring features, as inactivation of MEN1, VHL, TSC1/2 genes, and hyperactivation of the PI3K/mTOR pathway...
January 10, 2018: Journal of Endocrinology
https://www.readbyqxmd.com/read/29250314/a-case-of-long-survival-insulinoma-with-multiple-neuroendocline-tumour-type-1-controlled-by-multimodal-therapy
#9
Masahiro Shiihara, Wataru Izumo, Ryota Higuchi, Takehisa Yazawa, Shuichiro Uemura, Toru Furukawa, Masakazu Yamamoto
Insulinomas with multiple neuroendocrine tumour type 1 (MEN1) sometimes have metachronous or recurrent tumours. However, the treatment for these tumours is controversial, and published reports regarding multimodal therapy for insulinomas are limited. We report a 73-year-old woman with recurrent insulinoma with MEN1 successfully controlled by multimodal therapy. She had several complications, and poor performance status. Her hypoglycaemia did not improve after 6-month octreotide LAR; as such, she underwent enucleation of the pancreatic tumour...
December 2017: Journal of Surgical Case Reports
https://www.readbyqxmd.com/read/29239038/gastroenteropancreatic-neuroendocrine-neoplasms-selected-pathology-review-and-molecular-updates
#10
REVIEW
Siaw M Chai, Ian S Brown, M Priyanthi Kumarasinghe
Gastroenteropancreatic (GEP) neuroendocrine neoplasms can be broadly separated into well- and poorly differentiated categories. Tumours within each category have similarities in morphology and immunophenotype, but vary in grade, behaviour, molecular signature and responses to therapy. The aetiology of these differences is multifactorial. Site of origin, mucosal milieu and hereditary influences are some of the currently known factors. Given these differences, staging and grading systems continue to evolve, and the most recent World Health Organization classification of pancreatic neuroendocrine neoplasms reflects this by introducing a grade 3 neuroendocrine tumour category for morphologically well-differentiated tumours with an elevated Ki-67 proliferation index and/or mitotic count...
January 2018: Histopathology
https://www.readbyqxmd.com/read/29239037/well-differentiated-pancreatic-neuroendocrine-tumours-pannets-and-poorly-differentiated-pancreatic-neuroendocrine-carcinomas-pannecs-concepts-issues-and-a-practical-diagnostic-approach-to-high-grade-g3-cases
#11
REVIEW
Aatur D Singhi, David S Klimstra
With increasing accessibility and advancements in abdominal imaging modalities, the incidence of pancreatic neuroendocrine neoplasms has increased steadily during the past few decades. By definition, neuroendocrine neoplasms of the pancreas show neuroendocrine differentiation, but they represent a broad and heterogeneous group of neoplasms with diverse clinical and pathological characteristics. The majority of pancreatic neuroendocrine neoplasms can be classified as well-differentiated pancreatic neuroendocrine tumours (PanNETs) or poorly differentiated pancreatic neuroendocrine carcinomas (PanNECs)...
January 2018: Histopathology
https://www.readbyqxmd.com/read/29229497/advanced-neuroendocrine-tumours-of-the-small-intestine-and-pancreas-clinical-developments-controversies-and-future-strategies
#12
REVIEW
Christoph J Auernhammer, Christine Spitzweg, Martin K Angele, Stefan Boeck, Ashley Grossman, Svenja Nölting, Harun Ilhan, Thomas Knösel, Julia Mayerle, Martin Reincke, Peter Bartenstein
In this Review, we discuss clinical developments and controversies in the treatment of neuroendocrine tumours (NETs) that are relevant for clinicians and clinical researchers. We describe advances in genetics, blood-based biomarkers, functional imaging, and systemic therapy of advanced NETs and discuss results of recent phase 3 studies, systemic treatment of advanced disease with peptide receptor radionuclide therapy, biotherapy, chemotherapy, and molecularly targeted therapy, and the potential role of immunotherapy in the treatment of NETs...
December 8, 2017: Lancet Diabetes & Endocrinology
https://www.readbyqxmd.com/read/29205458/multi-disciplinary-management-of-refractory-insulinomas
#13
Emily Brown, Daniel Watkin, Jonathan Evans, Vincent Yip, Daniel J Cuthbertson
Insulinomas are predominantly benign (~90%), pancreatic neuroendocrine tumours characterised by hyperinsulinaemic hypoglycaemia. They usually present as a small (<2cm), well demarcated, solitary nodule that can arise in any part of the organ. Treatment of sporadic insulinomas is generally aimed at curative surgical resection with special consideration in genetic syndromes. Patients with significant hypoglycaemia can pose a difficult management challenge. In isolated cases where the patient is not medically fit for surgery or with metastatic spread, other treatment options are employed...
December 4, 2017: Clinical Endocrinology
https://www.readbyqxmd.com/read/29175872/notch-pathway-inhibition-targets-chemoresistant-insulinoma-cancer-stem-cells
#14
Ylenia Capodanno, Floryne O Buishand, Lisa Y Pang, Jolle Kirpensteijn, Jan A Mol, David J Argyle
Insulinomas (INS) are the most common neuroendocrine pancreatic tumours in humans and dogs. The long-term prognosis for malignant INS is still poor due to a low success rate of the current treatment modalities, particularly chemotherapy. A better understanding of the molecular processes underlying the development and progression of INS is required to develop novel targeted therapies. Cancer stem cells (CSCs) are thought to be critical for the engraftment and chemoresistance of many tumours, including INS. This study was aimed to characterise and target INS CSCs in order to develop novel targeted therapies...
November 24, 2017: Endocrine-related Cancer
https://www.readbyqxmd.com/read/29169836/-classification-of-pancreatic-neuroendocrine-tumours-changes-made-in-the-2017%C3%A2-who-classification-of-tumours-of-endocrine-organs-and-perspectives-for-the-future
#15
Jean-Yves Scoazec, Anne Couvelard
The WHO classification of the tumors of endocrine organs, published in July 2017, has introduced significant changes in the classification of pancreatic neuroendocrine tumors, the previous version of which has appeared in 2010, within the WHO classification of the tumors of the digestive system. The main change is the introduction of a new category of well-differentiated neoplasms, neuroendocrine tumors G3, in addition to the previous categories of neuroendocrine tumors G1 and G2. The differential diagnosis between neuroendocrine tumors G3 (well-differentiated) and neuroendocrine carcinomas (poorly-differentiated) might be difficult; the authors of the WHO classification therefore suggest the use of a number of immunohistochemical markers to facilitate the distinction between the two entities...
November 20, 2017: Annales de Pathologie
https://www.readbyqxmd.com/read/29161241/determination-of-an-optimal-response-cut-off-able-to-predict-progression-free-survival-in-patients-with-well-differentiated-advanced-pancreatic-neuroendocrine-tumours-treated-with-sunitinib-an-alternative-to-the-current-recist-defined-response
#16
Angela Lamarca, Jorge Barriuso, Matthew Kulke, Ivan Borbath, Heinz-Josef Lenz, Jean Luc Raoul, Neal J Meropol, Catherine Lombard-Bohas, James Posey, Sandrine Faivre, Eric Raymond, Juan W Valle
BACKGROUND: Sunitinib prolongs progression-free survival (PFS) in patients with advanced pancreatic neuroendocrine tumours (pNET). Response Evaluation Criteria in Solid Tumors (RECIST)-defined partial responses (PR; classically defined as ⩾30% size decrease from baseline) are infrequent. METHODS: Individual data of pNET patients from the phase II [NCT00056693] and pivotal phase III [NCT00428597] trials of sunitinib were analysed in this investigator-initiated, post hoc study...
November 21, 2017: British Journal of Cancer
https://www.readbyqxmd.com/read/29150545/tumour-diameter-is-not-reliable-for-management-of-non-secreting-pancreatic-neuroendocrine-tumours
#17
Logan Mills, Panagiotis Drymousis, Yogesh Vashist, Christoph Burdelski, Andreas Prachalias, Parthi Srinivasan, Krishna Menon, Corina Cotoi, Saboor Khan, Judith Cave, Thomas Armstrong, Martin O Weickert, Jakob Izbicki, Joerg Schrader, Andreja Frilling, John K Ramage, Raj Srirajaskanthan
Small non-functioning pancreatic NETs (pNETs) ≤2 cm can pose a management dilemma in terms of surveillance or resection. There is evidence to suggest that a surveillance approach can be considered since there are no significant radiological changes observed in lesions during long-term follow-up. However, other studies have suggested loco-regional spread can be present in ≤2 cm pNETs. The aim of this study was to characterise the prevalence of malignant features and identify any useful predictive variables in a surgically resected cohort of pNETs...
November 2017: Endocrine Connections
https://www.readbyqxmd.com/read/29137302/functional-and-non-functional-pancreatic-neuroendocrine-tumours-enets-or-ajcc-tnm-staging-system
#18
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
#19
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/29126671/treatment-challenges-in-and-outside-a-specialist-network-setting-pancreatic-neuroendocrine-tumours
#20
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
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