Gastrointestinal stromal tumours: ESMO–EURACAN–GENTURIS Clinical Practice Guidelines for diagnosis, treatment and follow-up

Casali, Paolo G.; Blay, Jean‐Yves; Abecassis, N.; Bajpai, Jyoti; Bauer, Sebastian; Biagini, R.; Bielack, Stefan; Bonvalot, Sylvie; Boukovinas, I.; Bovée, Judith V.M.G.; Boye, Kjetil; Brodowicz, Thomas; Buonadonna, Angela; Álava, Enrique de; Tos, Angelo Paolo Dei; Muro, Xavier García del; Dufresne, Armelle; Eriksson, Mikael; Феденко, А. А.; Ferraresi, Virginia; Ferrari, Andrea; Frezza, Anna Maria; Gasperoni, Silvia; Gelderblom, Hans; Gouin, F.; Grignani, Giovanni; Haas, Rick L.; Hassan, A B; Hindi, Nadia; Hohenberger, Peter; Joensuu, Heikki; Jones, Robin L.; Jungels, Christiane; Jutte, Paul C.; Kasper, Bernd; Kawai, Akira; Kopečková, Kateřina; Krákorová, Dagmar Adámková; Cesne, Axel Le; Grange, Franel le; Legius, Eric; Leithner, Andreas; López‐Pousa, Antonio; Martin‐Broto, Javier; Merimsky, Ofer; Messiou, Christina; Miah, Aisha; Mir, Olivier; Montemurro, Michael; Morosi, Carlo; Palmerini, Emanuela; Pantaleo, Maria Abbondanza; Piana, Raimondo; Piperno‐Neumann, Sophie; Reichardt, Peter; Rutkowski, Piotr; Safwat, Akmal; Sangalli, Claudia; Sbaraglia, Marta; Scheipl, Susanne; Schöffski, Patrick; Sleijfer, Stefan; Strauß, D.; Strauss, Sandra J.; Hall, Kirsten Sundby; Trama, Annalisa; Unk, Mojca; Sande, Michiel A.J. van de; Graaf, Winette T.A. van der; Houdt, Winan J. van; Frébourg, Thierry; Gronchi, Alessandro; Stacchiotti, Silvia

doi:10.1016/j.annonc.2021.09.005

Cited by 314 publications

(281 citation statements)

References 72 publications

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“…Patients with SFTs should be managed within sarcoma reference centers, by a dedicated multidisciplinary team with a pathologist, radiologist, surgical oncologist, radiation oncologist, and medical oncologist who are familiar with the nuances of this disease [ 65 , 66 , 67 , 68 ]. Each case has to be discussed in a specialized multidisciplinary tumor board (MTB) to determine the best individualized therapeutic strategy.…”

Section: Therapeutic Options For Sftmentioning

confidence: 99%

“…Metachronous (disease-free interval >1 year), resectable lung metastases without extrapulmonary disease may be managed with surgery as standard treatment applied to sarcoma, if complete excision of all lesions is feasible. Surgery, ablations, or RT of extrapulmonary metastases may also be an option in highly selected cases [ 65 , 66 , 67 ].…”

Section: Therapeutic Options For Sftmentioning

confidence: 99%

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Novel Therapeutic Options for Solitary Fibrous Tumor: Antiangiogenic Therapy and Beyond

Bernardi

Dufresne

Mishellany

et al. 2022

Cancers

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SFT is an ultrarare mesenchymal ubiquitous tumor, with an incidence rate <1 case/million people/year. The fifth WHO classification published in April 2020 subdivided SFT into three categories: benign (locally aggressive), NOS (rarely metastasizing), and malignant. Recurrence can occur in up to 10–40% of localized SFTs, and several risk stratification models have been proposed to predict the individual risk of metastatic relapse. The Demicco model is the most widely used and is based on age at presentation, tumor size, and mitotic count. Total en bloc resection is the standard treatment of patients with a localized SFT; in case of advanced disease, the clinical efficacy of conventional chemotherapy remains poor. In this review, we discuss new insights into the biology and the treatment of patients with SFT. NAB2–STAT6 oncogenic fusion, which is the pathognomonic hallmark of SFT, is supposedly involved in the overexpression of vascular endothelial growth factor (VEGF). These specific biological features encouraged the successful assessment of antiangiogenic drugs. Overall, antiangiogenic therapies showed a significant activity toward SFT in the advanced/metastatic setting. Nevertheless, these promising results warrant additional investigation to be validated, including randomized phase III trials and biological translational analysis, to understand and predict mechanisms of efficacy and resistance. While the therapeutic potential of immunotherapy remains elusive, the use of antiangiogenics as first-line treatment should be considered.

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Section: Therapeutic Options For Sftmentioning

confidence: 99%

Section: Therapeutic Options For Sftmentioning

confidence: 99%

Novel Therapeutic Options for Solitary Fibrous Tumor: Antiangiogenic Therapy and Beyond

Bernardi

Dufresne

Mishellany

et al. 2022

Cancers

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“…Gastrointestinal stromal tumors (GIST) are the most frequent sarcomas of the gastrointestinal tract [ 1 , 2 ]. They remain an exemplary model for targeted therapies within solid tumors: the presence of KIT or PDGFRA activating mutations (~85% of cases) [ 3 , 4 ] leads to high efficiency of the tyrosine kinase inhibitors (imatinib, sunitinib, regorafenib, and avapritinib) [ 5 , 6 ] currently marketed.…”

Section: Introductionmentioning

confidence: 99%

“…However, primary or secondary resistance occurs in all cases and a critical goal is to develop new therapies able to overcome it. Adjuvant imatinib results in lower relapse rates [ 7 , 8 ] and increases overall survival [ 8 ] in localized stages treated by surgery [ 2 ]; it is currently recommended for patients at intermediate or high risk of metastatic relapse, according to the AFIP classification [ 2 , 9 ]. Nevertheless, classifications based on clinicopathological features, such as AFIP, remain imperfect and need to be improved [ 5 , 10 , 11 , 12 , 13 ].…”

Section: Introductionmentioning

confidence: 99%

CSPG4 Expression in GIST Is Associated with Better Prognosis and Strong Cytotoxic Immune Response

Nonneville

Finetti

Picard

et al. 2022

Cancers

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The treatment of gastrointestinal stromal tumors (GIST) must be improved through the development of more reliable prognostic factors and of therapies able to overcome imatinib resistance. The immune system represents an attractive tool. CSPG4, a cell surface proteoglycan, emerged as a potential therapeutic target for immune therapy in different cancers, including cell therapy based on CSPG4-specific chimeric antigen receptor (CAR)-redirected cytokine-induced killer lymphocytes (CSPG4-CAR.CIKs) in sarcomas. CSPG4 expression has never been studied in GIST. We analyzed CSPG4 mRNA expression data of 309 clinical GIST samples profiled using DNA microarrays and searched for correlations with clinicopathological and immune features. CSPG4 expression, higher in tumors than normal digestive tissues, was heterogeneous across tumors. High expression was associated with AFIP low-risk, gastric site, and localized stage, and independently with longer postoperative disease-free survival (DFS) in localized stage. The correlations between CSPG4 expression and immune signatures highlighted a higher anti-tumor immune response in “CSPG4-high” tumors, relying on both the adaptive and innate immune system, in which the boost of NK cells by CSPG4-CAR.CIKs might be instrumental, eventually combined with immune checkpoint inhibitors. In conclusion, high CSPG4 expression in GIST is associated with better DFS and offers an immune environment favorable to a vulnerability to CAR.CIKs.

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“…GIST, which occurs most commonly in the stomach and small intestine at middle and old age ( Joensuu et al, 2001 ; Miettinen et al, 2002 ; von Mehren and Joensuu, 2018 ), has an incidence of 10 per million populations per year generally and comprises around 20% of the soft tissue sarcomas ( Ducimetiere et al, 2011 ; Joensuu et al, 2013 ). Standard therapies for GIST confirmed by immunohistology are as the following: for the resectable GISTs without metastasis, surgical resection is the first choice; and for the unresectable, metastatic, or recurrent GISTs, administration of tyrosine kinase inhibitors (TKIs) is the primary approach ( Nishida et al, 2008a ; Li J. et al, 2017 ; Casali et al, 2022 ).…”

Section: Introductionmentioning

confidence: 99%

Noncoding RNAs in Drug Resistance of Gastrointestinal Stromal Tumor

Guo

Sun

et al. 2022

Front. Cell Dev. Biol.

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Gastrointestinal stromal tumor (GIST) is the most common mesenchymal tumor in the gastrointestinal tracts and a model for the targeted therapy of solid tumors because of the oncogenic driver mutations in KIT and PDGDRA genes, which could be effectively inhibited by the very first targeted agent, imatinib mesylate. Most of the GIST patients could benefit a lot from the targeted treatment of this receptor tyrosine kinase inhibitor. However, more than 50% of the patients developed resistance within 2 years after imatinib administration, limiting the long-term effect of imatinib. Noncoding RNAs (ncRNAs), the non-protein coding transcripts of human, were demonstrated to play pivotal roles in the resistance of various chemotherapy drugs. In this review, we summarized the mechanisms of how ncRNAs functioning on the drug resistance in GIST. During the drug resistance of GIST, there were five regulating mechanisms where the functions of ncRNAs concentrated: oxidative phosphorylation, autophagy, apoptosis, drug target changes, and some signaling pathways. Also, these effects of ncRNAs in drug resistance were divided into two aspects. How ncRNAs regulate drug resistance in GIST was further summarized according to ncRNA types, different drugs and categories of resistance. Moreover, clinical applications of these ncRNAs in GIST chemotherapies concentrated on the prognostic biomarkers and novel therapeutic targets.

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Gastrointestinal stromal tumours: ESMO–EURACAN–GENTURIS Clinical Practice Guidelines for diagnosis, treatment and follow-up

Cited by 314 publications

References 72 publications

Novel Therapeutic Options for Solitary Fibrous Tumor: Antiangiogenic Therapy and Beyond

Novel Therapeutic Options for Solitary Fibrous Tumor: Antiangiogenic Therapy and Beyond

CSPG4 Expression in GIST Is Associated with Better Prognosis and Strong Cytotoxic Immune Response

Noncoding RNAs in Drug Resistance of Gastrointestinal Stromal Tumor

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