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gastrointestinal stromal tumour

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https://www.readbyqxmd.com/read/28433494/patterns-of-care-and-clinical-outcomes-in-primary-oesophageal-gastrointestinal-stromal-tumours-gist-a-retrospective-study-of-the-french-sarcoma-group-fsg
#1
F Duffaud, P Meeus, F Bertucci, J-B Delhorme, E Stoeckle, N Isambert, E Bompas, J Gagniere, O Bouché, M Toulmonde, S Salas, J-Y Blay, S Bonvalot
BACKGROUND: Oesophageal GIST (ESOGIST) are very rare tumours requiring special consideration regarding diagnosis, surgical management, and perioperative treatment. METHODS: A retrospective study was conducted across 9 centres in the French Sarcoma Group (FSG) to characterize all patients in the years 2000-2014. RESULTS: Seventeen patients (pts) with primary localized ESOGIST were identified, with median age 69 years (36-81) and 11 females...
April 1, 2017: European Journal of Surgical Oncology
https://www.readbyqxmd.com/read/28432162/conventional-transanal-excision-for-a-very-low-gastrointestinal-stromal-tumour
#2
Georgios C Sotiropoulos, Paraskevas Stamopoulos, Stylianos Kykalos, Nikolaos Machairas
Gastrointestinal stromal tumour (GIST) represents a rare tumour entity, which has been more intensively investigated during the last decade. The rectum as the primary site of GIST is even uncommon. The space constraints in the pelvis renders optimal oncological surgery demanding and proximity of these lesions to the anal sphincter enhances the potential morbidity of any radical surgery. We herein report on a young patient with a >5 cm rectal GIST localised at 1 cm from the anorectal junction.
April 21, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28416775/volatile-organic-compounds-in-gastrointestinal-stromal-tumour-tissue-originating-from-patient-derived-xenografts
#3
Renata Wawrzyniak, Agnieszka Woźniak, Yemarshet Gebreyohannes, Bartosz Dykcik, Patrick Schöffski, Michal Markuszewski
Gastrointestinal stromal tumours (GIST) are the most common mesenchymal neoplasms of the gastrointestinal tract, localized mainly in the stomach or small intestine. The metabolomic signature of GIST signature associated with various mutations in KIT gene remains undiscovered and unexplored. The main aim of this pilot study was to determine and compare metabolomic profiles in GIST xenograft models with different genetic background. Metabolomic profiling using gas chromatography coupled with mass spectrometry followed by both univariate and multivariate statistical analyses was applied to select metabolites which differentiate the GIST models studied...
April 18, 2017: Journal of Breath Research
https://www.readbyqxmd.com/read/28403213/m-copa-suppresses-endolysosomal-kit-akt-oncogenic-signalling-through-inhibiting-the-secretory-pathway-in-neoplastic-mast-cells
#4
Yasushi Hara, Yuuki Obata, Keita Horikawa, Yasutaka Tasaki, Kyohei Suzuki, Takatsugu Murata, Isamu Shiina, Ryo Abe
Gain-of-function mutations in Kit receptor tyrosine kinase result in the development of a variety of cancers, such as mast cell tumours, gastrointestinal stromal tumours (GISTs), acute myeloid leukemia, and melanomas. The drug imatinib, a selective inhibitor of Kit, is used for treatment of mutant Kit-positive cancers. However, mutations in the Kit kinase domain, which are frequently found in neoplastic mast cells, confer an imatinib resistance, and cancers expressing the mutants can proliferate in the presence of imatinib...
2017: PloS One
https://www.readbyqxmd.com/read/28399879/splenosis-with-lower-gastrointestinal-bleeding-mimicking-colonical-gastrointestinal-stromal-tumour
#5
Shuo-Meng Xiao, Rui Xu, Xiao-Li Tang, Zhi Ding, Ji-Man Li, Xiang Zhou
BACKGROUND: Splenosis refers to the heterotopic transplantation of splenic tissue following splenic trauma or splenectomy. Splenosis is typically asymptomatic and is often identified incidentally. CASE PRESENTATION: We report a case of splenosis with colon and stomach invasion presenting as lower gastrointestinal bleeding and mimicking colonic gastrointestinal stromal tumour (GIST). The importance of suspicion for splenosis in patients with a history of splenic injury should be highlighted...
April 11, 2017: World Journal of Surgical Oncology
https://www.readbyqxmd.com/read/28386296/high-risk-gastrointestinal-stromal-tumour-gist-and-synovial-sarcoma-display-similar-angiogenic-profiles-a-nude-mice-xenograft-study
#6
Francisco Giner, Isidro Machado, Jose Antonio Lopez-Guerrero, Empar Mayordomo-Aranda, Antonio Llombart-Bosch
BACKGROUND: Gastrointestinal stromal tumour (GIST) is the most common primary mesenchymal tumour of the gastrointestinal tract. Spindle cell monophasic synovial sarcoma (SS) can be morphologically similar. Angiogenesis is a major factor for tumour growth and metastasis. Our aim was to compare the angiogenic expression profiles of high-risk GIST and spindle cell monophasic SS by histological, immunohistochemical and molecular characterisation of the neovascularisation established between xenotransplanted tumours and the host during the initial phases of growth in nude mice...
2017: Ecancermedicalscience
https://www.readbyqxmd.com/read/28384968/paraneoplastic-hypoglycaemia-a-rare-manifestation-of-pelvic-gastrointestinal-stromal-tumour
#7
Ashish Singhal, Rahat Hadi, Kiranpreet Mehrotra, Shivani Rastogi, Shakeel Masood
Non-Islet Cell Tumour Induced Hypoglycaemia (NICTH), presenting with recurrent fasting hypoglycaemia is a very rare paraneoplastic syndrome. It usually presents with large metastatic mesenchymal tumours. NICTH secondary to Gastrointestinal Stromal Tumour (GIST) is even rarer. Diagnosis of NICTH is based on the low serum insulin level, low serum concentrations of Insulin Like Growth Factor (IGF-I) and IGF binding protein- III (IGFBP-III) in combination with elevated concentrations of pro-IGF-II. Various Immunohistochemical (IHC) markers are integral to diagnosis of GIST namely 2-deoxyglucose-6-phosphate phosphatase -1(DOG-1), Cluster Differentiation 34 (CD 34), Cluster Differentiation 117 (CD117)...
February 2017: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28371039/surgery-for-gastrointestinal-stromal-tumours-in-australia-and-new-zealand-results-from-a-bi-national-audit
#8
Rajeev Parameswaran, Ross H Roberts, Wendy A Brown, Ahmad Aly, George Kiroff, Krishna Epari, Andrew D MacCormick, Iain G Thomson, Sarah K Thompson
No abstract text is available yet for this article.
April 2017: ANZ Journal of Surgery
https://www.readbyqxmd.com/read/28361439/cost-effectiveness-analysis-of-regorafenib-for-gastrointestinal-stromal-tumour-gist-in-germany
#9
David Tamoschus, Katja Draexler, Jane Chang, Christopher Ngai, Matthew Madin-Warburton, Ashley Pitcher
BACKGROUND: No study has compared the cost-effectiveness of active treatment options for unresectable or metastatic gastrointestinal stromal tumours in patients who progressed on or are intolerant to prior treatment with imatinib and sunitinib. The aim of this study was to estimate the cost-effectiveness of regorafenib compared to imatinib rechallenge in this setting in Germany. METHODS: Hazard ratios for progression-free (PFS) and overall survival (OS) with regorafenib versus imatinib rechallenge were estimated by indirect comparison...
March 30, 2017: Clinical Drug Investigation
https://www.readbyqxmd.com/read/28351781/geis-guidelines-for-gastrointestinal-sarcomas-gist
#10
REVIEW
Andrés Poveda, Xavier García Del Muro, Jose Antonio López-Guerrero, Ricardo Cubedo, Virginia Martínez, Ignacio Romero, César Serrano, Claudia Valverde, Javier Martín-Broto
Gastrointestinal stromal sarcomas (GISTs) are the most common mesenchymal tumours originating in the digestive tract. They have a characteristic morphology, are generally positive for CD117 (c-kit) and are primarily caused by activating mutations in the KIT or PDGFRA genes(1). On rare occasions, they occur in extravisceral locations such as the omentum, mesentery, pelvis and retroperitoneum. GISTs have become a model of multidisciplinary work in oncology: the participation of several specialties (oncologists, pathologists, surgeons, molecular biologists, radiologists…) has forested advances in the understanding of this tumour and the consolidation of a targeted therapy, imatinib, as the first effective molecular treatment in solid tumours...
March 2, 2017: Cancer Treatment Reviews
https://www.readbyqxmd.com/read/28334729/slug-transcription-factor-a-pro-survival-and-prognostic-factor-in-gastrointestinal-stromal-tumour
#11
Olli-Pekka Pulkka, Bengt Nilsson, Maarit Sarlomo-Rikala, Peter Reichardt, Mikael Eriksson, Kirsten Sundby Hall, Eva Wardelmann, Aki Vehtari, Heikki Joensuu, Harri Sihto
BACKGROUND: The SLUG transcription factor has been linked with the KIT signalling pathway that is important for gastrointestinal stromal tumour (GIST) tumourigenesis. Its clinical significance in GIST is unknown. METHODS: Influence of SLUG expression on cell proliferation and viability were investigated in GIST48 and GIST882 cell lines. The association between tumour SLUG expression in immunohistochemistry and recurrence-free survival (RFS) was studied in two clinical GIST series, one with 187 patients treated with surgery alone, and another one with 313 patients treated with surgery and adjuvant imatinib...
March 23, 2017: British Journal of Cancer
https://www.readbyqxmd.com/read/28284172/clinical-characteristics-and-treatment-outcome-in-a-large-multicentre-observational-cohort-of-pdgfra-exon-18-mutated-gastrointestinal-stromal-tumour%C3%A2-patients
#12
Sheima Farag, Neeta Somaiah, Haesun Choi, Birthe Heeres, Wei-Lien Wang, Hester van Boven, Petra Nederlof, Robert Benjamin, Winette van der Graaf, Dirk Grunhagen, Pieter A Boonstra, Anna K L Reyners, Hans Gelderblom, Neeltje Steeghs
PURPOSE: Patients, platelet-derived growth factor receptor alpha (PDGFRA) D842V-mutated gastrointestinal stromal tumours (GISTs) are known for their insensitivity to imatinib. However, in clinical practice responses have been observed in some patients. We describe the natural history and treatment outcomes in a cohort of PDGFRA exon 18 mutated GIST patients. PATIENTS AND METHODS: A retrospective cohort study was conducted in PDGFRA exon 18 mutation GIST patients treated in six expert centres in the Netherlands and the United States...
March 8, 2017: European Journal of Cancer
https://www.readbyqxmd.com/read/28281485/laparoscopic-resection-of-duodenal-gastrointestinal-stromal-tumour
#13
Tammy Zioni, Vitaliy Dizengof, Boris Kirshtein
Only a few studies have revealed using laparoscopic technique with limited resection of gastrointestinal stromal tumour (GIST) of the duodenum. A 68-year-old man was admitted to the hospital due to upper gastrointestinal (GI) bleeding. Evaluation revealed an ulcerated, bleeding GI tumour in the second part of the duodenum. After control of bleeding during gastroduodenoscopy, he underwent a laparoscopic wedge resection of the area. During 1.5 years of follow-up, the patient is disease free, eats drinks well, and has regained weight...
April 2017: Journal of Minimal Access Surgery
https://www.readbyqxmd.com/read/28271873/gastrointestinal-stromal-tumour-detection-with-somatostatin-receptor-imaging-68-ga-ha-dotatate-pet-ct
#14
Arthur J A T Braat, Roel Goldschmeding, Lodewijk A A Brosens, Menno R Vriens, Bart de Keizer
No abstract text is available yet for this article.
March 2017: Lancet Oncology
https://www.readbyqxmd.com/read/28270683/max-inactivation-is-an-early-event-in-gist-development-that-regulates-p16-and-cell-proliferation
#15
Inga-Marie Schaefer, Yuexiang Wang, Cher-Wei Liang, Nacef Bahri, Anna Quattrone, Leona Doyle, Adrian Mariño-Enríquez, Alexandra Lauria, Meijun Zhu, Maria Debiec-Rychter, Susanne Grunewald, Jaclyn F Hechtman, Armelle Dufresne, Cristina R Antonescu, Carol Beadling, Ewa T Sicinska, Matt van de Rijn, George D Demetri, Marc Ladanyi, Christopher L Corless, Michael C Heinrich, Chandrajit P Raut, Sebastian Bauer, Jonathan A Fletcher
KIT, PDGFRA, NF1 and SDH mutations are alternate initiating events, fostering hyperplasia in gastrointestinal stromal tumours (GISTs), and additional genetic alterations are required for progression to malignancy. The most frequent secondary alteration, demonstrated in ∼70% of GISTs, is chromosome 14q deletion. Here we report hemizygous or homozygous inactivating mutations of the chromosome 14q MAX gene in 16 of 76 GISTs (21%). We find MAX mutations in 17% and 50% of sporadic and NF1-syndromic GISTs, respectively, and we find loss of MAX protein expression in 48% and 90% of sporadic and NF1-syndromic GISTs, respectively, and in three of eight micro-GISTs, which are early GISTs...
March 8, 2017: Nature Communications
https://www.readbyqxmd.com/read/28208869/mature-cystic-teratoma-with-co-existent-mucinous-cystadenocarcinoma-in-the-same-ovary-a-diagnostic-dilemma
#16
Sanjeet Roy, Sramana Mukhopadhayay, Mayank Gupta, Anuradha Chandramohan
Mature cystic teratoma co-existing with a mucinous cystadenocarcinoma is an infrequently encountered entity with only a handful of cases reported till date. The possibilities in such a case are either a malignant transformation of a benign teratoma into adenocarcinoma or a collision tumor between a mature cystic teratoma and a mucinous tumour of either a primary ovarian surface epithelial-stromal origin or a secondary from a primary gastrointestinal tract tumour. The importance of distinguishing between the two entities has significant bearing on subsequent therapeutic management...
December 2016: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28203166/gastrointestinal-stromal-tumour-with-synchronous-bone-metastases-a-case-report-and-literature-review
#17
Philippe Rochigneux, Lénaig Mescam-Mancini, Delphine Perrot, Erwan Bories, Laurence Moureau-Zabotto, Anthony Sarran, Jérôme Guiramand, François Bertucci
Gastrointestinal stromal tumours (GISTs) are mesenchymal tumours of the digestive tract, derived from Cajal interstitial cells. Bone metastases are very rare, and there is no consensus regarding their treatment. Here, we present the unusual case of a 66-year-old man with a gastric GIST with synchronous bone and liver metastases, fully documented at the pathological and molecular levels with a KIT exon 11 mutation. After 9 months of imatinib, the scanner showed a 33% partial response of target lesions. We also review the literature and describe the characteristics, treatment, and outcome of all cases previously reported...
January 2017: Case Reports in Oncology
https://www.readbyqxmd.com/read/28199021/successful-treatment-of-a-metastatic-gastrointestinal-stromal-tumour-in-a-dog-with-toceranib-phosphate-palladia
#18
J W Elliott, F Swinbourne, A Parry, L Baines
A ten-year-old, female-entire English springer spaniel presented with a large intra-abdominal mass but no other clinical signs. Gastrointestinal stromal tumour of the caecum with widespread abdominal metastasis was confirmed. Treatment with toceranib phosphate resulted in complete response, despite the absence of exon-8 or exon-11 c-kit mutation. There was no clinical evidence of tumour recurrence nine months after diagnosis.
February 15, 2017: Journal of Small Animal Practice
https://www.readbyqxmd.com/read/28170370/development-of-a-gene-panel-for-next-generation-sequencing-of-clinically-relevant-mutations-in-cell-free-dna-from-cancer-patients
#19
Umberto Malapelle, Clara Mayo de-Las-Casas, Danilo Rocco, Monica Garzon, Pasquale Pisapia, Nuria Jordana-Ariza, Maria Russo, Roberta Sgariglia, Caterina De Luca, Francesco Pepe, Alejandro Martinez-Bueno, Daniela Morales-Espinosa, María González-Cao, Niki Karachaliou, Santiago Viteri Ramirez, Claudio Bellevicine, Miguel Angel Molina-Vila, Rafael Rosell, Giancarlo Troncone
BACKGROUND: When tumour tissue is unavailable, cell-free DNA (cfDNA)can serve as a surrogate for genetic analyses. Because mutated alleles in cfDNA are usually below 1%, next-generation sequencing (NGS)must be narrowed to target only clinically relevant genes. In this proof-of-concept study, we developed a panel to use in ultra-deep sequencing to identify such mutations in cfDNA. METHODS: Our panel ('SiRe') covers 568 mutations in six genes (EGFR, KRAS, NRAS, BRAF, cKIT and PDGFRα)involved in non-small-cell lung cancer (NSCLC), gastrointestinal stromal tumour, colorectal carcinoma and melanoma...
March 14, 2017: British Journal of Cancer
https://www.readbyqxmd.com/read/28104721/gastrointestinal-stromal-tumour-gist-presenting-as-a-strangulated-inguinal-hernia-with-small-bowel-obstruction
#20
Marco Massani, Giovanni Capovilla, Cesare Ruffolo, Nicolò Bassi
Gastrointestinal stromal tumours (GISTs) can arise everywhere along the gastrointestinal (GI) tract. Their presentation in unusual locations should always be taken into account. A 74-year-old man referred to the emergency department for small bowel obstruction caused by an incarcerated inguinal hernia. A CT scan showed a mesenchymal tumour originating from the herniated bowel loop and a mass in the ascending colon. Laparoscopic resection of the mass and laparoscopic right hemicolectomy were performed. The histology showed a ruptured GIST arising from the herniated small bowel and a high-grade dysplasia villous adenoma of the right colon...
January 19, 2017: BMJ Case Reports
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