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Treatment of gastrointestinal stromal tumours in paediatric and young adult patients with sunitinib: a multicentre case series
Gastrointestinal stromal tumours (GIST) are rarely encountered mesenchymal tumours of the gastrointestinal tract (1.5 people per 100,000/year) that are even more rarely seen in paediatric patients (1 –2% of all...
Source: BMC Cancer - November 6, 2017 Category: Cancer & Oncology Authors: Piotr Rutkowski, Heather Magnan, Alexander J. Chou and Charlotte Benson Tags: Research article Source Type: research

Cancers, Vol. 12, Pages 730: Gastrointestinal Stromal Tumours (GIST) in Young Adult (18 –40 Years) Patients: A Report from the Dutch GIST Registry
In conclusion, YA GISTs are not similar to typical adult GISTs and also differ from paediatric GISTs, as described in the literature. In this series, we found a relatively high percentage of small intestine GIST, emergency presentation, 25% non-KIT/PDGFRA mutations and a relatively good survival.
Source: Cancers - March 19, 2020 Category: Cancer & Oncology Authors: Nikki S. IJzerman Cas Drabbe Dide den Hollander Mahmoud Mohammadi Hester van Boven Ingrid M.E. Desar Hans Gelderblom Dirk J. Gr ünhagen An K.L. Reyners Max M. van Noesel Ron H.J. Mathijssen Neeltje Steeghs Winette T.A. van der Graaf Tags: Article Source Type: research

THZ1 targeting CDK7 suppresses c-KIT transcriptional activity in gastrointestinal stromal tumours
Gastrointestinal stromal tumours (GISTs) are the most common mesenchymal tumours of the gastrointestinal tract and are characterized by activating mutations of c-KIT or PDGFRa receptor tyrosine kinases (RTKs)....
Source: Cell Communication and Signaling - September 8, 2022 Category: Molecular Biology Authors: Jianyi Sun, Qiang Zhang, Xiangfei Sun, Anwei Xue, Xiaodong Gao and Kuntang Shen Tags: Research Source Type: research

Gastrointestinal stromal tumours: from KIT to succinate dehydrogenase
The discovery of activating mutations in the tyrosine kinase receptor genes KIT and PDGFRA has led to the development of effective targeted therapies for gastrointestinal stromal tumours (GISTs). Specific genotypes, in part, predict the response to treatment with tyrosine kinase inhibitors. However, ~10% of GISTs lack such mutations (often referred to as ‘wild‐type’ GISTs). Recent insights into the biology of ‘wild‐type’ GISTs have resulted in clinically significant subclassification of this heterogeneous group of tumours, a large subset of which are now known to represent succinate dehydrogenase‐deficient GI...
Source: Histopathology - November 28, 2013 Category: Pathology Authors: Leona A Doyle, Jason L Hornick Tags: Review Source Type: research

Endoscopic ultrasound-guided fine needle tissue acquisition biopsy samples do not allow a reliable proliferation assessment of gastrointestinal stromal tumours
Current prognostication of gastrointestinal stromal tumours is validated on/applies to resected tumours, mainly because surgery is recommended whenever possible. However, pre-treatment prognostication is increasingly warranted, considering the follow-up strategy recently admitted for expectedly low-risk tumours and the possible distinctive molecular features/spontaneous regression of some small cases.
Source: Digestive and Liver Disease - January 14, 2015 Category: Gastroenterology Authors: Riccardo Ricci, Gaia Chiarello, Fabia Attili, Lorenzo Fuccio, Sergio Alfieri, Roberto Persiani, Salvatore Di Pietro, Maurizio Martini, Guido Costamagna, Luigi M. Larocca, Alberto Larghi Tags: Digestive Endoscopy Source Type: research

EGFR gene mutation in gastrointestinal stromal tumours
ConclusionsWe first established that GISTs carrying EGFR mutation are relatively benign tumours. Although EGFR mutations were rarely present in GIST, EGFR seems to play a significant role in the development and progression of GIST.
Source: Histopathology - July 24, 2017 Category: Pathology Authors: Shan ‐Shan Shi, Nan Wu, Yan He, Xue Wei, Qiu‐Yuan Xia, Xuan Wang, Sheng‐Bin Ye, Rui Li, Qiu Rao, Xiao‐Jun Zhou Tags: Original Article Source Type: research

Role of immune microenvironment in gastrointestinal stromal tumours
ConclusionsPD‐L1 expression was associated with increased size and mitoses. High CD8+ or CD3+ TIL counts were associated with decreased PD‐L1/IDO+ GIST size. PD‐L1 and IDO could be significant in GIST tumour biology, which invites consideration of immunotherapy as a potential treatment option.
Source: Histopathology - November 21, 2017 Category: Pathology Authors: Andrew M Blakely, Andres Matoso, Pallavi A Patil, Ross Taliano, Jason T Machan, Thomas J Miner, Kara A Lombardo, Murray B Resnick, Li ‐Juan Wang Tags: Original Article Source Type: research

Biology of gastrointestinal stromal tumour and mechanisms of imatinib resistance
Abstract: The introduction of imatinib therapy for GISTs has represented a major breakthrough of oncology in the last decade, improving dramatically the prognosis of GIST patients. The discovery of an oncogenic event (kinase mutations) having a major predictive value underlines the need for a paradigm of classification combining thorough pathological examination and molecular analysis. The response to therapy with imatinib is indeed determined by the type of mutation in kinase genes. Genetic analysis should be therefore performed in all cases deserving such a therapy. The study of the resistance has enabled us to discover ...
Source: Diagnostic Histopathology - May 13, 2013 Category: Pathology Authors: Luigi Tornillo Tags: Mini-Symposium: Pathology of Gastrointestinal Stromal Tumours Source Type: research

Gastrointestinal stromal tumours in patients with neurofibromatosis type 1: A case report and retrospective review of 72 cases
We report a case of NF1‐associated GIST, and discuss the clinical and pathological data of 71 reported cases from the PubMed/MEDLINE database over the past two decades. The clinical characteristics, histopathology, biological behaviour, immunohistochemistry, phenotype and gene mutation status of GIST in NF1 are carefully reviewed. A total of 72 cases (35 females and 37 males; median age: 53 years) was included in this study. More than 80 per cent of lesions were located in the small intestine, most of which were multicentric (63.89 per cent), and the median diameter of the tumours was 4 cm (range: 0.3–20 cm). The p...
Source: Surgical Practice - July 15, 2013 Category: Surgery Authors: Ke Xiao, Xin‐Yu Tan, Hui‐Huan Tang, Shi Chang, Jun Zhou, Xue‐Jun Gong Tags: CASE REPORT Source Type: research

Genomic index predicts clinical outcome of intermediate-risk gastrointestinal stromal tumours, providing a new inclusion criterion for imatinib adjuvant therapy
Imatinib mesylate is the front-line targeted therapy for gastrointestinal stromal tumours (GISTs). Patient’s eligibility to adjuvant imatinib after primary tumour resection is currently based on histological and clinical risk assessment. While therapeutic options are clear for the very-low, low and high-risk subpopulations, no standard is actually available for the tumours classified as intermediate. Since we recently validated genomic index (GI), a measure of the level of genomic alterations, as a strong predictor of clinical outcome in GIST, we asked whether it could also represent a novel prognostic factor for the intermediate subgroup.
Source: European Journal of Cancer - November 6, 2014 Category: Cancer & Oncology Authors: Lydia Lartigue, Agnès Neuville, Pauline Lagarde, Céline Brulard, Piotr Rutkowski, Paolo Dei Tos, Eva Wardelmann, Maria Debiec-Rychter, Antoine Italiano, Jean-Michel Coindre, Frédéric Chibon Source Type: research

Prognostic characteristics of duodenal gastrointestinal stromal tumours
ConclusionThe prognosis of duodenal GISTs is similar to that of small intestinal GISTs.
Source: British Journal of Surgery - May 18, 2015 Category: Surgery Authors: Q. Zhang, C.‐H. Shou, J.‐R. Yu, W.‐L. Yang, X.‐S. Liu, H. Yu, Y. Gao, Q.‐Y. Shen, Z.‐C. Zhao Tags: Original article Source Type: research

Sporadic diffuse segmental interstitial cell of Cajal hyperplasia harbouring two gastric Gastrointestinal Stromal Tumours (GIST) mimicking hereditary GIST syndromes
Conclusion We describe a diffuse form of sporadic ICC hyperplasia harbouring multifocal GISTs, mimicking diffuse ICC hyperplasia in hereditary GIST syndromes. Detection of somatic c-KIT exon 11 mutation ruled out a hereditary disorder.
Source: International Journal of Surgery Case Reports - October 23, 2015 Category: Surgery Source Type: research

Molecular and morphological correlation in gastrointestinal stromal tumours (GISTs): an update and primer
Gastrointestinal stromal tumours (GISTs) are a commonly encountered tumour in routine practice. In the main, the morphology of spindle, epithelioid or mixed are well recognised along with mutations of c-kit. However, there are other genes that are mutated resulting in characteristic clinicopathological correlations. GISTs harbouring platelet-derived growth factor receptor α (PDGFRα) gene mutations lead to a typical morphological constellation of findings: gastric and omental location, gross tumour that is cystic and haemorrhagic, composed of epithelioid, plasmacytoid cells exhibiting pleomorphism, low mitotic c...
Source: Journal of Clinical Pathology - August 16, 2016 Category: Pathology Authors: Chetty, R., Serra, S. Tags: Editor's choice, Molecular genetics, Immunology (including allergy) Review Source Type: research

Radiofrequency ablation versus resection for resectable liver metastases of gastrointestinal stromal tumours: Results from three national centres in China
ConclusionsFor patients with resectable liver metastases from GISTs after receiving pre-operative TKI treatment, compared with resection, ablation seemed to be associated with shorter progression-free survival, but RFA offered comparable overall survival, and the post- procedure morbidity and lengths of stay were significantly lower. With complete ablation of the targeted tumours, our results suggest that RFA is an acceptable option in selected patients.
Source: Clinics and Research in Hepatology and Gastroenterology - November 16, 2018 Category: Gastroenterology Source Type: research

Gastrointestinal stromal tumours of stomach: Robot-assisted excision with the da Vinci Surgical System regardless of size and location site
Conclusion: Our experience suggests a positive role of the robot da Vinci in getting gastric GIST removal with a conservative approach, regardless of size and location site. Comparative studies with a greater number of patients are necessary for a more robust assessment.
Source: Journal of Minimal Access Surgery - March 11, 2019 Category: Surgery Authors: Niccol & #242; Furbetta Matteo Palmeri Simone Guadagni Gregorio Di Franco Desir & #233;e Gianardi Saverio Latteri Emanuele Marciano Andrea Moglia Alfred Cuschieri Giulio Di Candio Franco Mosca Luca Morelli Source Type: research