keyword
https://read.qxmd.com/read/38431792/fulminant-ectopic-cushing-s-syndrome-caused-by-metastatic-small-intestine-neuroendocrine-tumour-a-case-report-and-review-of-the-literature
#1
B Alliet, C Severi, T Veekmans, J Cuypers, H Topal, C M Deroose, T Roskams, M Bex, J Dekervel
Cushing's syndrome (CS) secondary to adrenocorticotropic hormone (ACTH) producing tumours is a severe condition with a challenging diagnosis. Ectopic ACTH-secretion often involves neuroendocrine tumours (NET) in the respiratory tract. ACTH-secreting small intestine neuro-endocrine tumours (siNET) are extremely rare entities barely reported in literature. This review is illustrated by the case of a 75-year old woman with fulminant ectopic CS caused by a ACTH-secreting metastatic siNET. Severe hypokalemia, fluid retention and refractory hypertension were the presenting symptoms...
2024: Acta Gastro-enterologica Belgica
https://read.qxmd.com/read/38294638/the-effectiveness-and-safety-of-proton-beam-radiation-therapy-in-children-and-young-adults-with-central-nervous-system-cns-tumours-a-systematic-review
#2
REVIEW
Jayne S Wilson, Caroline Main, Nicky Thorp, Roger E Taylor, Saimma Majothi, Pamela R Kearns, Martin English, Madhumita Dandapani, Robert Phillips, Keith Wheatley, Barry Pizer
BACKGROUND: Central nervous system (CNS) tumours account for around 25% of childhood neoplasms. With multi-modal therapy, 5-year survival is at around 75% in the UK. Conventional photon radiotherapy has made significant contributions to survival, but can be associated with long-term side effects. Proton beam radiotherapy (PBT) reduces the volume of irradiated tissue outside the tumour target volume which may potentially reduce toxicity. Our aim was to assess the effectiveness and safety of PBT and make recommendations for future research for this evolving treatment...
January 31, 2024: Journal of Neuro-oncology
https://read.qxmd.com/read/38224403/presentation-and-outcomes-of-paediatric-craniopharyngioma-in-the-west-of-scotland-a-25-year-experience
#3
JOURNAL ARTICLE
Xuan Ye Chanel Kuah, Angela K Lucas-Herald, Sarah McCarrison, Roisin Boyle, Claire Adey, Anthony Amato-Watkins, Parameswaran Bhattathiri, Emer Campbell, Fiona Cowie, Liam Dorris, Ben Fulton, Diana Mcintosh, Dermot Murphy, Milind Ronghe, Roddy O'Kane, Lorraine Todd, Meharpal Sangra, Jairam Sastry, Eoghan Millar, Samih Hassan, M Guftar Shaikh
PURPOSE: Craniopharyngiomas can be aggressive leading to significant complications and morbidity. It is not clear whether there are any predictive factors for incidence or outcomes. Our aim was therefore to record the incidence, presentation, characteristics and progression of paediatric craniopharyngiomas in the West of Scotland. METHOD: Retrospective case note review for children diagnosed with paediatric craniopharyngiomas at the Royal Hospital for Children Glasgow, from 1995 to 2021 was conducted...
January 15, 2024: Journal of Neuro-oncology
https://read.qxmd.com/read/38153453/vhl-trends-and-insight-into-a-multi-modality-interdisciplinary-approach-for-management-of-central-nervous-system-hemangioblastoma
#4
REVIEW
S A Matloob, D Paraskevopoulos, S M O'Toole, W Drake, N Plowman, N Foroglou
Von Hippel-Lindau (VHL) is a multi-system disease which results in significant morbidity from central nervous system (CNS) involvement as well as ocular, renal and neuro-endocrine effects. Haemangioblastomas of the CNS present a number of challenges. The natural history of these lesions is varied, as is the size and location within the CNS. Whilst surgery is considered the mainstay of treatment and best chance at curing these lesions, this is also often associated with significant risks due to the anatomical location of these lesions, most commonly the posterior fossa and spinal cord...
2023: Acta Neurochirurgica. Supplement
https://read.qxmd.com/read/37897617/intra-arterial-peptide-receptor-radionuclide-therapy-for-neuro-endocrine-tumour-liver-metastases-an-in-patient-randomised-controlled-trial-lutia
#5
JOURNAL ARTICLE
S C Ebbers, M W Barentsz, D M V de Vries-Huizing, M W J Versleijen, E G Klompenhouwer, M E T Tesselaar, M P M Stokkel, T Brabander, J Hofland, A Moelker, R S van Leeuwaarde, M L J Smits, A J A T Braat, M G E H Lam
PURPOSE: Peptide receptor radionuclide therapy (PRRT) using [177 Lu]Lu-DOTATATE has been shown to effectively prolong progression free survival in grade 1-2 gastroenteropancreatic neuroendocrine tumours (GEP-NET), but is less efficacious in patients with extensive liver metastases. The aim was to investigate whether tumour uptake in liver metastases can be enhanced by intra-arterial administration of [177 Lu]Lu-DOTATATE into the hepatic artery, in order to improve tumour response without increasing toxicity...
October 28, 2023: European Journal of Nuclear Medicine and Molecular Imaging
https://read.qxmd.com/read/37434651/clinical-presentation-of-sporadic-and-hereditary-pheochromocytoma-paraganglioma
#6
JOURNAL ARTICLE
Sofia Maria Lider Burciulescu, Caren Randon, Frederic Duprez, Wouter Huvenne, David Creytens, Kathleen B M Claes, Robin de Putter, Guy T'Sjoen, Corin Badiu, Bruno Lapauw
Pheochromocytomas (PHEO) and paragangliomas (PGL) can occur sporadic or within genetic predisposition syndromes. Despite shared embryology, there are important differences between PHEO and PGL. The aim of this study was to describe the clinical presentation and disease characteristics of PHEO/PGL. A retrospective analysis of consecutively registered patients diagnosed with or treated for PHEO/PGL in a tertiary care centre was performed. Patients were compared according to anatomic location (PHEO vs PGL) and genetic status (sporadic vs hereditary)...
January 1, 2023: Endocr Oncol
https://read.qxmd.com/read/37359931/appendiceal-goblet-cell-carcinoma-role-of-cytoreductive-surgery-crs-and-hyperthermic-intraperitoneal-chemotherapy-hipec
#7
REVIEW
M Gaillard, P Van Eyken, G Verswijvel, K Van der Speeten
Goblet cell carcinoma (GCC) encompasses a separate entity in appendiceal neoplasms with mixed glandular and neuro-endocrine pathological features. GCC mostly presents as an acute appendicitis duo to luminal obstruction or as an incidental finding on the surgical appendectomy specimen. In case of tumour perforation or presence of other risk factors, guidelines suggest additional treatment with a completing right hemicolectomy or cytoreductive surgery (CRS) with hyperthermic intraperitoneal chemotherapy (HIPEC)...
June 2023: Indian Journal of Surgical Oncology
https://read.qxmd.com/read/36696572/rapid-onset-obesity-hypothalamic-dysfunction-hypoventilation-and-autonomic-dysregulation-syndrome%C3%A2-neuro-endocrine-tumours-rohhad-net-case-series-and-learning-points
#8
Katherine Hawton, Tom Hilliard, Simon C Langton-Hewer, Christine Burren, Elizabeth C Crowne, Julian P Hamilton-Shield, Dinesh Giri
OBJECTIVES: Rapid-onset obesity with hypoventilation, hypothalamic dysfunction, autonomic dysregulation (ROHHAD) is a rare syndrome associated with high morbidity and mortality. Diagnosis is often challenging. We describe three cases of ROHHAD with heterogeneous presentations but some consistent clinical features, including hyperprolactinaemia at diagnosis. We highlight when the diagnosis of ROHHAD should be considered at an early stage. CASE PRESENTATION: All three patients presented between 4 and 6 years old with rapid-onset obesity...
January 25, 2023: Journal of Pediatric Endocrinology & Metabolism: JPEM
https://read.qxmd.com/read/36215066/the-role-of-potassium-channels-in-tumours-of-the-gastrointestinal-tract-a-focus-on-the-human-ether-%C3%A3-go-go-related-gene-1-channels
#9
JOURNAL ARTICLE
Annarosa Arcangeli, Claudia Duranti, Jessica Iorio, Elena Lastraioli
Potassium channels are often dysregulated in tumours of the gastrointestinal (GI) tract. Among them, the voltage-dependent channel KV 11.1, also known as human ether-à-go-go related gene 1 (hERG1), is frequently expressed in tumours and precancerous lesions of the GI tract. In precancerous lesions, hERG1 behaves as a progression factor, contributing to identifying those patients whose lesions can progress towards true cancers. In advanced cancers, such as colorectal and pancreatic cancer, a high hERG1 expression represents a negative prognostic factor, contributing to identifying high risk patients...
October 10, 2022: Journal of Physiology
https://read.qxmd.com/read/35592897/pituitary-apoplexy-in-an-adolescent-male-with-macroprolactinoma-presenting-as-middle-cerebral-artery-territory-infarction
#10
JOURNAL ARTICLE
Pankaj Agrawal, Sally Newbold, Ayisha Al Busaidi, Ritika R Kapoor, Nick Thomas, Simon Jb Aylwin, Charles R Buchanan, Ved Bhushan Arya
Pituitary apoplexy typically presents with acute headache, vomiting, visual disturbance and confusion. We report a rare presentation of ischaemic stroke due to pituitary apoplexy. A 16.5-year-old male presented with reduced Glasgow Coma Score, slurred speech, right-sided hemiparesis, and bi-temporal hemianopia. Magnetic Resonance Imaging of brain showed a large haemorrhagic sellar/suprasellar mass and an area of cortical T2/FLAIR hyperintensity with corresponding diffusion restriction in middle cerebral artery territory...
May 16, 2022: Annals of Pediatric Endocrinology & Metabolism
https://read.qxmd.com/read/34599569/evaluating-the-accuracy-of-three-international-guidelines-in-identifying-the-risk-of-malignancy-in-pancreatic-cysts-a-retrospective-analysis-of-a-surgical-treated-population
#11
JOURNAL ARTICLE
A Vanden Bulcke, J Jaekers, H Topal, D Vanbeckevoort, V Vandecaveye, T Roskams, B A Weynand, J Dekervel, E Van Cutsem, H van Malenstein, C Verslype, W Laleman, S van der Merwe
BACKGROUND AND STUDY AIMS: The international consensus Fukuoka guideline (Fukuoka ICG), The European evidence-based guideline on pancreatic cystic neoplasms (European EBG) and the American Gastroenterological Association institute guideline on the diagnosis and management of asymptomatic neoplastic pancreatic cysts (AGA IG) are 3 frequently cited guidelines for the risk stratification of neoplastic pancreatic cysts. The aim of this study was to assess the accuracy of detecting malignant cysts by strictly applying these guidelines retrospectively to a cohort of surgically resected pancreatic cysts...
July 2021: Acta Gastro-enterologica Belgica
https://read.qxmd.com/read/34118528/the-incidence-treatment-and-survival-of-patients-with-rare-types-of-rectal-malignancies-in-the-netherlands-a-population-based-study-between-1989-and-2018
#12
JOURNAL ARTICLE
Jan M van Rees, Marloes A G Elferink, Pieter J Tanis, Johannes H W de Wilt, Jacobus W A Burger, Cornelis Verhoef
AIM: To describe the incidence, treatment and survival of patients with rare types of rectal malignancies in the Netherlands. METHODS: Data of patients with rectal malignancies diagnosed in the Netherlands between 1989 and 2018 were retrieved from the Netherlands Cancer Registry and grouped according to the RARECARE cancer list. Age-standardised incidence rates were calculated using the European Standard Rate. The Joinpoint Regression Program was used for analysing trends and joinpoints and for the estimation of annual percentage changes (APCs)...
July 2021: European Journal of Cancer
https://read.qxmd.com/read/33835149/the-incidence-of-major-subtypes-of-primary-brain-tumors-in-adults-in-england-1995-2017
#13
JOURNAL ARTICLE
Hiba A Wanis, Henrik Møller, Keyoumars Ashkan, Elizabeth A Davies
BACKGROUND: Primary brain tumors are a complex heterogenous group of benign and malignant tumors. Reports on their occurrence in the English population by sex, age, and morphological subtype and on their incidence are currently not available. Using data from the National Cancer Registration and Analysis Service (NCRAS), the incidence of adult primary brain tumor by major subtypes in England will be described. METHODS: Data on all adult English patients diagnosed with primary brain tumor between 1995 and 2017, excluding spinal, endocrinal, and other CNS tumors, were extracted from NCRAS...
August 2, 2021: Neuro-oncology
https://read.qxmd.com/read/33263561/transition-of-young-adults-with-endocrine-and-metabolic-diseases-the-transend-cohort
#14
JOURNAL ARTICLE
Enora Le Roux, Florence Menesguen, Isabelle Tejedor, Marc Popelier, Marine Halbron, Pauline Faucher, Sabine Malivoir, Graziella Pinto, Juliane Léger, Stephane Hatem, Michel Polak, Christine Poitou, Philippe Touraine
OBJECTIVE: The transition from paediatric to adult medicine involves risks of poor patient outcomes and of significant losses of patients to follow up. The research aimed to analyse the implementation in an initial cohort of patients of a new programme of transition to adult care based on a case management approach. DESIGN: A longitudinal study of the case management approach to transition, initiated in a university hospital in France in September 2016. METHODS: Patients with the endocrine or metabolic disease diagnosed during childhood and transferred to adult care were included...
January 2021: Endocrine Connections
https://read.qxmd.com/read/33016048/primary-small-cell-neuroendocrine-carcinoma-of-the-bladder-case-report-and-literature-review
#15
REVIEW
Valerio Olivieri, Valentina Fortunati, Luca Bellei, Massimo Massarelli, Gabriele Ruggiero, Danilo Abate, Nicoletta Serra, Daniele Griffa, Flavio Forte, Emanuele Corongiu
BACKGROUND: Neuroendocrine tumours (NET) are extremely rare and aggressive. Although they commonly affect intestine, many organs may be involved such as pancreas, lung or urinary tract. Bladder is rarely involved. Actually, two main forms of bladder NET have been described: small-cell and large-cell. The first one is considered highly agressive since it shows poor oncologic outcomes being mainly diagnosed at advanced stage: the second one is extremely rare and equally aggressive. CASE REPORT: A 78-years-old Caucasian male presented to our facility for lower urinary tract symptoms and gross hematuria recently occurred...
October 2, 2020: Archivio Italiano di Urologia, Andrologia
https://read.qxmd.com/read/32454200/primary-lumbar-paraganglioma-clinical-radiologic-surgical-and-histopathologic-characteristics-from-a-case-series-of-13-patients
#16
JOURNAL ARTICLE
Francesco Fiorini, José Pedro Lavrador, Francesco Vergani, Ranjeev Bhangoo, Richard Gullan, Zita Reisz, Safa Al-Sarraj, Keyoumars Ashkan
BACKGROUND: Paragangliomas are uncommon neuroendocrine tumors, rarely occurring in the lumbar spine. Primary lumbar paragangliomas are prominently vascularized, can present variably, and pose both diagnostic and surgical challenges. We report on a large case series with long-term follow-up and intraoperative footage to characterize the natural history, diagnostic approach, and operative approach to this rare surgical disease. METHODS: This is a single-center, retrospective cohort study including all patients with histologically confirmed primary lumbar paraganglioma treated at our tertiary neurosurgical center between 1997 and 2018...
October 2020: World Neurosurgery
https://read.qxmd.com/read/32155405/colonic-goblet-cell-carcinoid-rarity-of-a-rarity-a-case-report-and-review-of-literature
#17
REVIEW
Abdalla Saad Abdalla, Kashuf Arooj Khan, Ahmed Shah, Amira Asaad, Victoria Salter, Marina Barron, Soad Eldruki, Victoria Salih, Salem Omar Alowami
Goblet cell carcinoids (GCC) are extremelyrare neuroendocrine tumours, and characterised by their unique combination of two types of cancer cells âÃÂ" neuroendocrine (carcinoid) and epithelial (adeno-carcinoma). In spite of the fact that GCC is regarded as Neuro-Endocrine Tumour (NET), it does not illicit carcinoid syndrome. GCC usually arises in the appendix and accounting for less than 14% of all appendiceal tumours.Primary extra-appendiceal GCC have been reported as stomach, duodenum, small intestine, colon and rectum...
January 2020: Chirurgia
https://read.qxmd.com/read/32031064/clinical-and-translational-research-challenges-in-neuroendocrine-tumours
#18
REVIEW
Jorge Barriuso, Angela Lamarca
Neuroendocrine tumours (NETs) represent a range of neoplasms that may arise from any (neuro)endocrine cell situated in any part of the human body. As any other rare diseases, NETs face several difficulties in relation to research. This review will describe some of the main challenges and proposed solutions faced by researchers with expertise in rare malignancies. Some of the most common challenges in clinical and translational research are enumerated in this review, covering aspects from clinical, translational and basic research...
2020: Current Medicinal Chemistry
https://read.qxmd.com/read/31683040/communication-and-management-of-incidental-pathology-in-1-214-consecutive-appendicectomies-a-cohort-study
#19
JOURNAL ARTICLE
Ned Kinnear, Bridget Heijkoop, Eliza Bramwell, Alannah Frazzetto, Amy Noll, Prajay Patel, Derek Hennessey, Greg Otto, Christopher Dobbins, Tarik Sammour, James Moore
BACKGROUND: Important incidental pathology requiring further action is commonly found during appendicectomy, macro- and microscopically. We aimed to determine whether the acute surgical unit (ASU) model improved the management and disclosure of these findings. METHODS: An ASU model was introduced at our institution on 01/08/2012. In this retrospective cohort study, all patients undergoing appendicectomy 2.5 years before (Traditional group) or after (ASU group) this date were compared...
December 2019: International Journal of Surgery
https://read.qxmd.com/read/31445889/a-rational-approach-to-postoperative-surveillance-for-resected-non-functional-pancreatic-neuro-endocrine-tumours
#20
JOURNAL ARTICLE
M Feretis, T Wang, E Ghorani, A Balakrishnan, S J Harper, A Jah, E L Huguet, R K Praseedom, S-S Liau
BACKGROUND: Non-functional pancreatic neuroendocrine tumours (NF-PNETs) are rare and have highly variable outcomes. Current guidelines recommend surveillance for NF-PNETs <2 cm. Patients who ultimately have surgical resection are at risk of disease recurrence, and data to support postoperative surveillance protocols are lacking. The aims of this study were to i) identify post-operative predictors of recurrence and ii) risk stratify patients at risk of recurrence. METHODS: Consecutive patients who underwent surgery for NF-PNETs between 2002 and 2015 were identified retrospectively...
October 2019: Pancreatology: Official Journal of the International Association of Pancreatology (IAP) ... [et Al.]
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