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gastrointestinal stromal tumour

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https://www.readbyqxmd.com/read/28723416/unravelling-the-pharmacologic-opportunities-and-future-directions-for-targeted-therapies-in-gastro-intestinal-cancers-part-2-neuroendocrine-tumours-hepatocellular-carcinoma-and-gastro-intestinal-stromal-tumours
#1
REVIEW
Cindy Neuzillet, Louis de Mestier, Benoît Rousseau, Olivier Mir, Mohamed Hebbar, Hemant M Kocher, Philippe Ruszniewski, Christophe Tournigand
Until the 1990s, cytotoxic chemotherapy has been the cornerstone of medical therapy for gastrointestinal (GI) cancers. Better understanding of the cancer cell molecular biology has led to the therapeutic revolution of targeted therapies, i.e. monoclonal antibodies or small molecule inhibitors directed against proteins that are specifically overexpressed or mutated in cancer cells. These agents, being more specific to cancer cells, were expected to be less toxic than conventional cytotoxic agents. However, their effects have sometimes been disappointing, due to intrinsic or acquired resistance mechanisms, or to an activity restricted to some tumour settings, illustrating the importance of patient selection and early identification of predictive biomarkers of response to these therapies...
July 16, 2017: Pharmacology & Therapeutics
https://www.readbyqxmd.com/read/28720692/primary-greater-omental-gist-presenting-with-acute-intra-abdominal-haemorrhage
#2
Shariful Islam, Devin Hosein, Vinoo Bheem, Patrick Harnarayan
Primary omental gastrointestinal stromal tumours (GISTs) are classified as extra-GISTs with a reported incidence of <1% of all GISTs. There are only a few cases in the English literature reporting omental GIST with none reporting acute intra-abdominal haemorrhage as a presenting feature. We report a case of a 67-year-old man who presented with haemorrhage from a primary omental GIST requiring emergency laparotomy.
July 18, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28694665/pancreas-preserving-distal-duodenectomy-a-versatile-operation-for-a-range-of-infra-papillary-pathologies
#3
W Kyle Mitchell, Pradeep F Thomas, Abed M Zaitoun, Adam J Brooks, Dileep N Lobo
AIM: To investigate the range of pathologies treated by pancreas preserving distal duodenectomy (PPDD) and present the outcome of follow-up. METHODS: Neoplastic lesions of the duodenum are treated conventionally by pancreaticoduodenectomy. Lesions distal to the major papilla may be suitable for a pancreas-preserving distal duodenectomy, potentially reducing morbidity and mortality. We present our experience with this procedure. Selective intraoperative duodenoscopy assessed the relationship of the papilla to the lesion...
June 21, 2017: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/28662235/use-of-pd-1-targeting-macrophage-infiltration-and-ido-pathway-activation-in-sarcomas-a-phase-2-clinical-trial
#4
Maud Toulmonde, Nicolas Penel, Julien Adam, Christine Chevreau, Jean-Yves Blay, Axel Le Cesne, Emmanuelle Bompas, Sophie Piperno-Neumann, Sophie Cousin, Thomas Grellety, Thomas Ryckewaert, Alban Bessede, François Ghiringhelli, Marina Pulido, Antoine Italiano
Importance: There is a strong rationale for treating sarcomas with immunotherapy. Objective: To assess the efficacy and safety of programmed cell death protein 1 (PD-1) targeting in combination with metronomic chemotherapy in sarcomas. Design, Setting, and Participants: This was an open-label, multicenter, phase 2 study of 4 cohorts of patients with advanced soft-tissue sarcoma (STS), including leiomyosarcoma (LMS), undifferentiated pleomorphic sarcoma (UPS), other sarcomas (others), and gastrointestinal stromal tumor (GIST)...
June 29, 2017: JAMA Oncology
https://www.readbyqxmd.com/read/28660813/monophasic-synovial-sarcoma-of-the-greater-omentum-case-report-and-review-of-literature
#5
F J Alcalá Serrano, J R Hernández Hernández, T Montenegro Dámaso, E López-Tomassetti Fernández
Synovial sarcoma is a malignant spindle cell neoplasm normally arising from tissues around joints, bursa and tendon sheaths. Several reports involving the gastrointestinal tract, mainly the oesophagus and stomach, have been documented; however, the omentum remains an extremely unusual location. Monophasic type is composed exclusively of spindle cells arranged in fascicles. Establishing the correct diagnosis of these tumours could be challenging because of the similarities with gastrointestinal stromal tumours and other mesenchymal tumours with similar histology...
July 2017: Annals of the Royal College of Surgeons of England
https://www.readbyqxmd.com/read/28658787/gastric-glomus-tumour-misdiagnosed-as-gastric-carcinoid-an-unfamiliar-entity-with-aids-to-diagnosis-and-review-of-literature
#6
Tanush Vig, Mandeep Singh Bindra, Ramani Manoj Kumar, Suceena Alexander
Glomus tumour (GT) is a rare mesenchymal tumour of the stomach with Gastrointestinal Stromal Tumour (GIST), leiomyoma and schwannoma being far more common and comprising more than 90% of all gastric mesenchymal tumours. As glomus bodies are located in the peripheral parts of the human body, these tumours are peripherally located, classically the subungual region, hands, feet and trunk. While being evaluated for renal problems, a middle aged lady was incidentally found to have a gastric tumour. This was submucosal in location and was excised by a wedge resection and reported elsewhere as carcinoid tumour...
May 2017: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28622526/extragastrointestinal-stromal-tumour-of-the-lesser-omentum-a-case-report-and-literature-review
#7
I E Katsoulis, A Tzortzopoulou, P Tziakou, N Arnogiannaki, I Kostoglou-Athanassiou, G Lypas, I G Karaitianos
INTRODUCTION: Extragastrointestinal stromal tumours (EGISTs) are very uncommon compared to their gastrointestinal counterparts. Most of them originate from the intestinal mesentery and the omentum. CASE REPORT: A 70 year-old Caucasian woman presented with a bulky abdominal mass which on laparotomy was found to originate from the lesser omentum and was completely resected. Histological examination revealed spindle cells with severe pleomorphism and high mitotic activity...
June 8, 2017: International Journal of Surgery Case Reports
https://www.readbyqxmd.com/read/28584517/a-rare-case-of-mesenteric-gastrointestinal-stromal-tumor-presenting-as-acute-abdomen
#8
Anant Sadanand Ramani, Guruprasad Huske, Francisco P Noronha
Gastrointestinal stromal tumours (GIST) are rare tumours arising from mesenchyme of gastrointestinal tract and overexpress C-kit protein. Mainly seen in stomach and small bowel. Mesenteric GIST are rarely reported as they constitute less than 1% of total GIST. We here report such a rare case of GIST arising from mesentery of small bowel and presenting as acute abdomen. Good surgical clearance ensures good survival whereas incomplete resection results in a high incidence of recurrences with distant metastasis...
January 2017: Nigerian Journal of Surgery: Official Publication of the Nigerian Surgical Research Society
https://www.readbyqxmd.com/read/28557709/influence-of-cytochrome-p450-abc-and-slc-gene-polymorphisms-on-imatinib-therapy-outcome-of-patients-with-gastrointestinal-stromal-tumours-gist
#9
K Wasielewski, B Wasag, A Wozniak, J Pikiel, A Kowalik, C Osuch, E Bylina, J A Siedlecki, P Rutkowski, J Limon
The efficacy of imatinib-based therapy depends on the proteins involved in its metabolism and transportation. Therefore, the aim of our study was to investigate the possible correlation of selected P450, ABC and SLC polymorphic variants and the outcome of imatinib therapy. A total of 101 patients with advanced, KIT/PDGFRA(+) GIST treated with imatinib were enrolled to the study. DNA was extracted from peripheral blood samples and genotypes were determined by PCR-RFLP and direct sequencing. Deviation from the Hardy-Weinberg equilibrium was only observed for rs2740574...
2017: Folia Biologica (Praha)
https://www.readbyqxmd.com/read/28535156/phase-ii-study-of-neoadjuvant-imatinib-in-large-gastrointestinal-stromal-tumours-of-the-stomach
#10
Yukinori Kurokawa, Han-Kwang Yang, Haruhiko Cho, Min-Hee Ryu, Toru Masuzawa, Sook Ryun Park, Sohei Matsumoto, Hyuk-Joon Lee, Hiroshi Honda, Oh Kyoung Kwon, Takashi Ishikawa, Kyung Hee Lee, Kazuhito Nabeshima, Seong-Ho Kong, Toshio Shimokawa, Jeong-Hwan Yook, Yuichiro Doki, Seock-Ah Im, Seiichi Hirota, Seokyung Hahn, Toshirou Nishida, Yoon-Koo Kang
BACKGROUND: Gastrointestinal stromal tumours (GISTs) with high-risk features have poor prognosis even if adjuvant treatment is given. Neoadjuvant imatinib may increase the cure rate by shrinking large GISTs and preserve organ function. METHODS: We conducted an Asian multinational phase II study for patients with gastric GISTs ≥10 cm. Patients received neoadjuvant imatinib (400 mg/day) for 6-9 months. The primary end point was R0 resection rate. RESULTS: A total of 56 patients were enroled in this study...
June 27, 2017: British Journal of Cancer
https://www.readbyqxmd.com/read/28485054/egfr-gene-mutation-in-gastrointestinal-stromal-tumors
#11
Shan-Shan Shi, Nan Wu, Yan He, Xue Wei, Qiu-Yuan Xia, Xuan Wang, Sheng-Bin Ye, Rui Li, Qiu Rao, Xiao-Jun Zhou
AIMS: The aim of this study was to evaluate the mutation status of EGFR in gastrointestinal stromal tumours and its association with various clinicopathological variables as well as to further discuss its effects of EGFR mutations on tumor formation and progression. METHODS: A well-characterized cohort of 323 GISTs, obtained between 2010 and 2015 from the surgical pathology files of at the Department of Pathology of the Nanjing Jinling Hospital, was screened for mutations in exon 19 and 21 of the EGFR gene...
May 9, 2017: Histopathology
https://www.readbyqxmd.com/read/28465823/uk-clinical-practice-guidelines-for-the-management-of-gastrointestinal-stromal-tumours-gist
#12
Ian Judson, Ramesh Bulusu, Beatrice Seddon, Adam Dangoor, Newton Wong, Satvinder Mudan
BACKGROUND: Soft tissue sarcomas (STS) are rare tumours arising in mesenchymal tissues. Gastrointestinal stromal tumour (GIST) is the commonest STS and arises within the wall of the gastrointestinal (GI) tract. While most GISTs occur in the stomach they do occur in all parts of the GI tract. As with other STS, it is important that GISTs are managed by expert teams, to ensure consistent and optimal treatment, as well as recruitment to clinical trials, and the ongoing accumulation of further knowledge of the disease...
2017: Clinical Sarcoma Research
https://www.readbyqxmd.com/read/28448856/impact-of-a-risk-based-follow-up-in-patients-affected-by-gastrointestinal-stromal-tumour
#13
Lorenzo D'Ambrosio, Erica Palesandro, Paola Boccone, Francesco Tolomeo, Sara Miano, Danilo Galizia, Antonio Manca, Gabriele Chiara, Ilaria Bertotto, Filippo Russo, Delia Campanella, Tiziana Venesio, Dario Sangiolo, Ymera Pignochino, Dimitrios Siatis, Michele De Simone, Alessandro Ferrero, Alberto Pisacane, Angelo Paolo Dei Tos, Sandra Aliberti, Massimo Aglietta, Giovanni Grignani
BACKGROUND: Follow-up aims to precociously identify recurrences, metastases or treatment-related adverse events so as to undertake the appropriate therapy. Guidelines admit lack of knowledge on optimal surveillance schedule, but suggest follow-up based on experts' opinion and risk stratification. To identify the impact, if any, of regular follow-up, we interrogated our prospectively collected database whether early detection of recurrences affected both clinical management and, likely, the outcome...
April 24, 2017: European Journal of Cancer
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
#14
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...
June 2017: European Journal of Surgical Oncology
https://www.readbyqxmd.com/read/28432162/conventional-transanal-excision-for-a-very-low-gastrointestinal-stromal-tumour
#15
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
#16
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
#17
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
#18
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
#19
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
#20
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
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