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Pancreatic neuroendocrine tumour

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https://www.readbyqxmd.com/read/29675259/successful-neoadjuvant-peptide-receptor-radionuclide-therapy-for-an-inoperable-pancreatic-neuroendocrine-tumour
#1
Tiago Nunes da Silva, M L F van Velthuysen, Casper H J van Eijck, Jaap J Teunissen, J Hofland, Wouter W de Herder
Non-functional pancreatic neuroendocrine tumours (NETs) can present with advanced local or distant (metastatic) disease limiting the possibility of surgical cure. Several treatment options have been used in experimental neoadjuvant settings to improve the outcomes in such cases. Peptide receptor radionuclide therapy (PPRT) using beta emitting radiolabelled somatostatin analogues has been used in progressive pancreatic NETs. We report a 55-year-old female patient with a 12.8 cm pancreatic NET with significant local stomach and superior mesenteric vein compression and liver metastases...
2018: Endocrinology, Diabetes & Metabolism Case Reports
https://www.readbyqxmd.com/read/29629318/role-of-surgery-in-pancreatic-neuroendocrine-tumor
#2
REVIEW
Kai Pun Wong, Julian Shun Tsang, Brian Hung-Hin Lang
Pancreatic neuroendocrine tumours (PNETs) are rare. They are generally accepted to be slow-growing and have an indolent course. These tumours can be non-functioning or functioning, consisting of a biochemically heterogeneous group of tumours including insulinomas, gastrinomas, carcinoids and glucagonomas. Although surgery remains the mainstay of treatment, controversy still exists especially for non-functioning tumours <2 cm in size. Whether these should be resected or undergo intensive surveillance remains unclear...
February 2018: Gland Surgery
https://www.readbyqxmd.com/read/29615284/external-beam-radiotherapy-in-the-treatment-of-gastroenteropancreatic-neuroendocrine-tumours-a-systematic-review
#3
D L Chan, R Thompson, M Lam, N Pavlakis, J Hallet, C Law, S Singh, S Myrehaug
AIMS: External beam radiotherapy (EBRT) is infrequently used to treat gastroenteropancreatic neuroendocrine tumours (GEPNETS), with little published data to date. We carried out a systematic review to assess the activity of EBRT for GEPNETS. MATERIALS AND METHODS: Major databases were searched for papers including at least five patients treated with contemporary EBRT techniques. Eligible studies underwent dual independent review. The primary end points were response rate for lesions treated with definitive intent and recurrence-free survival for primary lesions treated with neoadjuvant or adjuvant intent...
March 31, 2018: Clinical Oncology: a Journal of the Royal College of Radiologists
https://www.readbyqxmd.com/read/29594173/acinar-cell-cystadenoma-a-rarity-in-advanced-von-hippel-lindau-disease-a-case-report
#4
Tim Fahlbusch, Andrea Tannapfel, Waldemar Uhl, Chris Braumann
Background: Von Hippel-Lindau (VHL) disease may occur at various localisations which can be both intra- and extrapancreatic as well as challenging to diagnose by medical imaging. Case Report: A positron emission tomography/magnetic resonance imaging in a 40-year-old woman was performed to monitor a haemangioblastoma. Additionally, it showed findings which were considered to be a pancreatic neuroendocrine tumour (pNET) and retroumbilical metastasis. The suspected metastasis was laparoscopically resected; however, pathological evaluation did not lead to a clear categorisation...
February 2018: Visceral Medicine
https://www.readbyqxmd.com/read/29550809/presenting-symptoms-and-delay-in-diagnosis-of-gastrointestinal-and-pancreatic-neuroendocrine-tumours
#5
Ron Basuroy, Cathy Bouvier, John Ramage, Maia Sissons, Alexandra Kent, Raj Srirajaskanthan
<br>Introduction The gastrointestinal tract and pancreas are common primary sites for neuroendocrine tumours (NETs). Patients often report long duration of non-specific symptoms in the year prior to diagnosis. The aims of this study were to establish pre-diagnosis patterns of symptoms. Secondly, to determine the time from onset of symptoms to NET diagnosis and understand the interaction with primary and secondary healthcare providers. Method A survey was designed on a web-based survey platform with the focus on patient symptoms prior to diagnosis and a screen for functional diarrhoea (Rome III criteria (C4))...
March 18, 2018: Neuroendocrinology
https://www.readbyqxmd.com/read/29526248/predictive-factors-of-endocrine-and-exocrine-insufficiency-after-resection-of-a-benign-tumour-of-the-pancreas
#6
Hélène Neophytou, Marc Wangermez, Elise Gand, Michel Carretier, Jérôme Danion, Jean-Pierre Richer
BACKGROUND: The aim of the present study is to evaluate the risk factors of endocrine and exocrine insufficiency occurring few years after pancreatic resections in a consecutive series of patients who underwent pancreatoduodenectomy (PD), left pancreatectomy (LP) or enucleation for benign neoplasms at a referral centre. METHODS: Pancreatic exocrine insufficiency (PEI) was defined by the onset of steatorrhea associated with weight loss, and endocrine insufficiency was determinate by fasting plasma glucose...
March 8, 2018: Annales D'endocrinologie
https://www.readbyqxmd.com/read/29518779/clinical-and-biomarker-evaluations-of-sunitinib-in-patients-with-grade-3-digestive-neuroendocrine-neoplasms
#7
Anna Pellat, Chantal Dreyer, Camille Couffignal, Thomas Walter, Catherine Lombard-Bohas, Patricia Niccoli, Jean François Seitz, Olivia Hentic, Thierry André, Romain Coriat, Sandrine Faivre, Magaly Zappa, Philippe Ruszniewski, Nicolas Pote, Anne Couvelard, Eric Raymond
BACKGROUND/AIMS: Angiogenesis is extensively developed in well-differentiated pancreatic neuroendocrine tumours (PanNET) where sunitinib was shown to prolong progression-free survival, leading to nationwide approval. However, clinical experience in patients with grade 3 gastroenteropancreatic neuroendocrine neoplasms (GEPNEN-G3) remains limited. This prospective phase II trial evaluated potential predictive biomarkers of sunitinib activity in patients with advanced GEPNEN-G3. METHODS: Sunitinib was given at a dose of 37...
March 8, 2018: Neuroendocrinology
https://www.readbyqxmd.com/read/29449689/current-and-emerging-therapies-for-pnets-in-patients-with-or-without-men1
#8
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...
April 2018: Nature Reviews. Endocrinology
https://www.readbyqxmd.com/read/29444910/the-neuroendocrine-phenotype-genomic-profile-and-therapeutic-sensitivity-of-gepnet-cell-lines
#9
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
#10
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
#11
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
#12
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
#13
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
#14
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
#15
REVIEW
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...
March 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
#16
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
#17
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
#18
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
#19
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/multidisciplinary-management-of-refractory-insulinomas
#20
REVIEW
Emily Brown, Daniel Watkin, Jonathan Evans, Vincent Yip, Daniel J Cuthbertson
Insulinomas are predominantly benign (~90%), pancreatic neuroendocrine tumours characterized by hyperinsulinaemic hypoglycaemia. They usually present as a small (<2 cm), well-demarcated, solitary nodule that can arise in any part of the organ. Treatment for 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
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