يعرض 1 - 10 نتائج من 28 نتيجة بحث عن '"Hohenberger, P."', وقت الاستعلام: 1.19s تنقيح النتائج
  1. 1
    دورية أكاديمية

    المصدر: European journal of cancer, vol. 190, pp. 112949

    الوصف: The aim of this study was to investigate the response rates of different extremity soft-tissue sarcoma subtypes (eSTS) after isolated limb perfusion (ILP), based on an international multi-centre study. The retrospective cohort comprised eSTS patients from 17 specialised ILP centres that underwent melphalan-based ILP, with or without recombinant human tumour necrosis factor (rhTNFα) (TM-ILP and M-ILP, respectively). Response was measured on imaging (magnetic resonance imaging) and/or clinical response, for which M-ILPs were excluded. A total of 1109 eSTS patients were included. The three most common histological subtypes were undifferentiated pleomorphic sarcoma (17%, n = 184), synovial sarcoma (16%, n = 175) and myxofibrosarcoma (8%, n = 87). rhTNFα was used in 93% (TM-ILP) and resulted in a significantly better overall response rate (ORR, p = 0.031) and complete responses (CR, p < 0.001) in comparison to M-ILP, without significant differences among histological subgroups. The ORR of TM-ILP was 68%, including 17% CR. Also, 80% showed progressive disease. Significantly higher response rates were shown for Kaposi sarcoma (KS) with 42% CR and 96% ORR (both p < 0.001), and significantly higher CR rates for angiosarcoma (AS, 45%, p < 0.001) and clear cell sarcoma (CCS, 31%, p = 0.049). ILP was followed by resection ≤ 6 months in 80% of the patients. The overall limb salvage rate was 88%, without significant differences among histological subgroups, but was significantly higher for ILP responders compared to non-responders (93% versus 76%, p < 0.001). ILP resulted in high response and LRS among all eSTS subtypes, however, with significant differences between subtypes with most promising results for KS, AS and CCS.

    وصف الملف: application/pdf

    العلاقة: info:eu-repo/semantics/altIdentifier/pmid/37453241; info:eu-repo/semantics/altIdentifier/eissn/1879-0852; info:eu-repo/semantics/altIdentifier/urn/urn:nbn:ch:serval-BIB_1346C91911814; https://serval.unil.ch/notice/serval:BIB_1346C9191181Test; urn:issn:0959-8049; https://serval.unil.ch/resource/serval:BIB_1346C9191181.P001/REF.pdfTest; http://nbn-resolving.org/urn/resolver.pl?urn=urn:nbn:ch:serval-BIB_1346C91911814Test

  2. 2
    دورية أكاديمية

    المساهمون: Jones, Robin

    جغرافية الموضوع: United States

    الوصف: PURPOSE: Primary or secondary mutations in KIT or platelet-derived growth factor receptor alpha (PDGFRA) underlie tyrosine kinase inhibitor resistance in most GI stromal tumors (GISTs). Avapritinib selectively and potently inhibits KIT- and PDGFRA-mutant kinases. In the phase I NAVIGATOR study (NCT02508532), avapritinib showed clinical activity against PDGFRA D842V-mutant and later-line KIT-mutant GIST. VOYAGER (NCT03465722), a phase III study, evaluated efficacy and safety of avapritinib versus regorafenib as third-line or later treatment in patients with unresectable or metastatic GIST. PATIENTS AND METHODS: VOYAGER randomly assigned patients 1:1 to avapritinib 300 mg once daily (4 weeks continuously) or regorafenib 160 mg once daily (3 weeks on and 1 week off). Primary end point was progression-free survival (PFS) by central radiology per RECIST version 1.1 modified for GIST. Secondary end points included objective response rate, overall survival, safety, disease control rate, and duration of response. Regorafenib to avapritinib crossover was permitted upon centrally confirmed disease progression. RESULTS: Four hundred seventy-six patients were randomly assigned (avapritinib, n = 240; regorafenib, n = 236). Median PFS was not statistically different between avapritinib and regorafenib (hazard ratio, 1.25; 95% CI, 0.99 to 1.57; 4.2 v 5.6 months; P = .055). Overall survival data were immature at cutoff. Objective response rates were 17.1% and 7.2%, with durations of responses of 7.6 and 9.4 months for avapritinib and regorafenib; disease control rates were 41.7% (95% CI, 35.4 to 48.2) and 46.2% (95% CI, 39.7 to 52.8). Treatment-related adverse events (any grade, grade ≥ 3) were similar for avapritinib (92.5% and 55.2%) and regorafenib (96.2% and 57.7%). CONCLUSION: Primary end point was not met. There was no significant difference in median PFS between avapritinib and regorafenib in patients with molecularly unselected, late-line GIST.

    وصف الملف: Print-Electronic; 3139; application/pdf

    العلاقة: Journal of Clinical Oncology, 2021, 39 (28), pp. 3128 - 3139; https://repository.icr.ac.uk/handle/internal/5486Test

  3. 3
    دورية أكاديمية

    المساهمون: Stacchiotti S., Miah A.B., Frezza A.M., Messiou C., Morosi C., Caraceni A., Antonescu C.R., Bajpai J., Baldini E., Bauer S., Biagini R., Bielack S., Blay J.Y., Bonvalot S., Boukovinas I., Bovee J.V.M.G., Boye K., Brodowicz T., Callegaro D., De Alava E., Deoras-Sutliff M., Dufresne A., Eriksson M., Errani C., Fedenko A., Ferraresi V., Ferrari A., Fletcher C.D.M., Garcia del Muro X., Gelderblom H., Gladdy R.A., Gouin F., Grignani G., Gutkovich J., Haas R., Hindi N., Hohenberger P., Huang P., Joensuu H., Jones R.L., Jungels C., Kasper B., Kawai A., Le Cesne A., Le Grange F., Leithner A., Leonard H., Lopez Pousa A., Martin Broto J., Merimsky O., Merriam P., Miceli R., Mir O., Molinari M., Montemurro M., Oldani G., Palmerini E., Pantaleo M.A., Patel S., Piperno-Neumann S., Raut C.P., Ravi V., Razak A.R.A., Reichardt P., Rubin B.P., Rutkowski P., Safwat A.A., Sangalli C., Sapisochin G., Sbaraglia M., Scheipl S., Schoffski P., Strauss D., Strauss S.J., Sundby Hall K., Tap W.D., Trama A., Tweddle A., van der Graaf W.T.A., Van De Sande M.A.J., Van Houdt W., van Oortmerssen G., Wagner A.J., Wartenberg M., Wood J., Zaffaroni N., Zimmermann C., Casali P.G., Dei Tos A.P., Gronchi A.

    الوصف: Epithelioid hemangioendothelioma (EHE) is an ultra-rare, translocated, vascular sarcoma. EHE clinical behavior is variable, ranging from that of a low-grade malignancy to that of a high-grade sarcoma and it is marked by a high propensity for systemic involvement. No active systemic agents are currently approved specifically for EHE, which is typically refractory to the antitumor drugs used in sarcomas. The degree of uncertainty in selecting the most appropriate therapy for EHE patients and the lack of guidelines on the clinical management of the disease make the adoption of new treatments inconsistent across the world, resulting in suboptimal outcomes for many EHE patients. To address the shortcoming, a global consensus meeting was organized in December 2020 under the umbrella of the European Society for Medical Oncology (ESMO) involving >80 experts from several disciplines from Europe, North America and Asia, together with a patient representative from the EHE Group, a global, disease-specific patient advocacy group, and Sarcoma Patient EuroNet (SPAEN). The meeting was aimed at defining, by consensus, evidence-based best practices for the optimal approach to primary and metastatic EHE. The consensus achieved during that meeting is the subject of the present publication.

    وصف الملف: STAMPA

    العلاقة: info:eu-repo/semantics/altIdentifier/pmid/34090171; info:eu-repo/semantics/altIdentifier/wos/WOS:000663044500059; volume:6; issue:3; firstpage:100170; lastpage:100179; numberofpages:10; journal:ESMO OPEN; http://hdl.handle.net/11585/864338Test; info:eu-repo/semantics/altIdentifier/scopus/2-s2.0-85108742417; https://www.sciencedirect.com/science/article/pii/S2059702921001307?via=ihubTest

  4. 4
    دورية أكاديمية

    المساهمون: Kang Y.-K., George S., Jones R.L., Rutkowski P., Shen L., Mir O., Patel S., Zhou Y., von Mehren M., Hohenberger P., Villalobos V., Brahmi M., Tap W.D., Trent J., Pantaleo M.A., Schoffski P., He K., Hew P., Newberry K., Roche M., Heinrich M.C., Bauer S.

    الوصف: PURPOSE Primary or secondary mutations in KIT or platelet-derived growth factor receptor alpha (PDGFRA) underlie tyrosine kinase inhibitor resistance in most GI stromal tumors (GISTs). Avapritinib selectively and potently inhibits KIT- and PDGFRA-mutant kinases. In the phase I NAVIGATOR study (NCT02508532), avapritinib showed clinical activity against PDGFRA D842V–mutant and later-line KIT-mutant GIST. VOYAGER (NCT03465722), a phase III study, evaluated efficacy and safety of avapritinib versus regorafenib as third-line or later treatment in patients with unresectable or metastatic GIST. PATIENTS AND METHODS VOYAGER randomly assigned patients 1:1 to avapritinib 300 mg once daily (4 weeks continuously) or regorafenib 160 mg once daily (3 weeks on and 1 week off). Primary end point was progression-free survival (PFS) by central radiology per RECIST version 1.1 modified for GIST. Secondary end points included objective response rate, overall survival, safety, disease control rate, and duration of response. Regorafenib to avapritinib crossover was permitted upon centrally confirmed disease progression. RESULTS Four hundred seventy-six patients were randomly assigned (avapritinib, n 5 240; regorafenib, n 5 236). Median PFS was not statistically different between avapritinib and regorafenib (hazard ratio, 1.25; 95% CI, 0.99 to 1.57; 4.2 v 5.6 months; P 5 .055). Overall survival data were immature at cutoff. Objective response rates were 17.1% and 7.2%, with durations of responses of 7.6 and 9.4 months for avapritinib and regorafenib; disease control rates were 41.7% (95% CI, 35.4 to 48.2) and 46.2% (95% CI, 39.7 to 52.8). Treatment-related adverse events (any grade, grade $ 3) were similar for avapritinib (92.5% and 55.2%) and regorafenib (96.2% and 57.7%). CONCLUSION Primary end point was not met. There was no significant difference in median PFS between avapritinib and regorafenib in patients with molecularly unselected, late-line GIST.

    وصف الملف: STAMPA

    العلاقة: info:eu-repo/semantics/altIdentifier/pmid/34343033; info:eu-repo/semantics/altIdentifier/wos/WOS:000708641200006; volume:39; issue:28; firstpage:3128; lastpage:3139; numberofpages:12; journal:JOURNAL OF CLINICAL ONCOLOGY; https://hdl.handle.net/11585/864328Test; info:eu-repo/semantics/altIdentifier/scopus/2-s2.0-85117739966

  5. 5
    دورية أكاديمية

    المساهمون: Alman B., Attia S., Baumgarten C., Benson C., Blay J.-Y., Bonvalot S., Breuing J., Cardona K., Casali P.G., van Coevorden F., Colombo C., Dei Tos A.P., Dileo P., Ferrari A., Fiore M., Frezza A.M., Garcia J., Gladdy R., Gounder M., Gronchi A., Haas R., Hackett S., Haller F., Hohenberger P., Husson O., Jones R.L., Judson I., Kasper B., Kawai A., Kogosov V., Lazar A.J., Maki R., Mathes T., Messiou C., Navid F., Nishida Y., Palassini E., Penel N., Pollock R., Pieper D., Portnoy M., Raut C.P., Roets E., Sandrucci S., Sbaraglia M., Stacchiotti S., Thornton K.A., van der Graaf W., van der Zande K., van Houdt W.J., Villalobos V., Wagner A.J., Wardelmann E., Wartenberg M., Watson S., Weiss A., Zafiropoulos N.

    الوصف: Desmoid tumor (DT; other synonymously used terms: Desmoid-type fibromatosis, aggressive fibromatosis) is a rare and locally aggressive monoclonal, fibroblastic proliferation characterised by a variable and often unpredictable clinical course. Previously surgery was the standard primary treatment modality; however, in recent years a paradigm shift towards a more conservative management has been introduced and an effort to harmonise the strategy amongst clinicians has been made. We present herein an evidence-based, joint global consensus guideline approach to the management of this disease focussing on: molecular genetics, indications for an active treatment, and available systemic therapeutic options. This paper follows a one-day consensus meeting held in Milan, Italy, in June 2018 under the auspices of the European Reference Network for rare solid adult cancers, EURACAN, the European Organisation for Research and Treatment of Cancer (EORTC) Soft Tissue and Bone Sarcoma Group (STBSG) as well as Sarcoma Patients EuroNet (SPAEN) and The Desmoid tumour Research Foundation (DTRF). The meeting brought together over 50 adult and pediatric sarcoma experts from different disciplines, patients and patient advocates from Europe, North America and Japan.

    العلاقة: info:eu-repo/semantics/altIdentifier/pmid/32004793; info:eu-repo/semantics/altIdentifier/wos/WOS:000514572100009; volume:127; issue:--; firstpage:96; lastpage:107; numberofpages:12; journal:EUROPEAN JOURNAL OF CANCER; http://hdl.handle.net/2318/1793788Test; info:eu-repo/semantics/altIdentifier/scopus/2-s2.0-85078263471

  6. 6
    دورية أكاديمية

    المساهمون: Judson, Ian

    الوصف: Sarcomas are a heterogeneous group of malignancies with mesenchymal lineage differentiation. The discovery of neurotrophic tyrosine receptor kinase (NTRK) gene fusions as tissue-agnostic oncogenic drivers has led to new personalized therapies for a subset of patients with sarcoma in the form of tropomyosin receptor kinase (TRK) inhibitors. NTRK gene rearrangements and fusion transcripts can be detected with different molecular pathology techniques, while TRK protein expression can be demonstrated with immunohistochemistry. The rarity and diagnostic complexity of NTRK gene fusions raise a number of questions and challenges for clinicians. To address these challenges, the World Sarcoma Network convened two meetings of expert adult oncologists and pathologists and subsequently developed this article to provide practical guidance on the management of patients with sarcoma harboring NTRK gene fusions. We propose a diagnostic strategy that considers disease stage and histologic and molecular subtypes to facilitate routine testing for TRK expression and subsequent testing for NTRK gene fusions.

    وصف الملف: Print-Electronic; 1517; application/pdf

    العلاقة: Annals of oncology : official journal of the European Society for Medical Oncology, 2020, 31 (11), pp. 1506 - 1517; https://repository.icr.ac.uk/handle/internal/4282Test

  7. 7

    المساهمون: Institut Català de la Salut, [Bonvalot S] Department of Surgical Oncology, Institut Curie, Paris University, Paris, France. [Rutkowski PL] Department of Soft Tissue/Bone Sarcoma and Melanoma, Maria Sklodowska-Curie National Research Institute of Oncology, Warsaw, Poland. [Thariat J] Department of Radiation Oncology, Centre François Baclesse, Caen, France. Department of Radiation Oncology, Centre Lacassagne, Nice, France. [Carrère S] Department of Surgical Oncology, Centre Regional De Lutte Contre Le Cancer Paul Lamarque, Montpellier, France. [Ducassou A] Department of Radiation Oncology, Institut Claudius Regaud (ICR), Institut Universitaire du Cancer de Toulouse-Oncopole (IUCT-O), Toulouse, France. [Sunyach MP] Department of Radiotherapy, Léon Bérard Cancer Center, Lyon, France. [Vergés R] Servei d’Oncologia Radioteràpica, Vall d'Hebron Hospital Universitari, Barcelona, Spain, Vall d'Hebron Barcelona Hospital Campus

    المصدر: International Journal of Radiation Oncology-Biology-Physics, 114(3), 422-432. ELSEVIER SCIENCE INC
    Scientia

    الوصف: Calidad de vida; Sarcoma de tejido blando localmente avanzado; Radioterapia Qualitat de vida; Sarcoma de teixit tou localment avançat; Radioteràpia Quality of life; Locally advanced soft-tissue sarcoma; Radiation therapy Purpose Act.In.Sarc (NCT02379845) demonstrated that the first-in-class radioenhancer NBTXR3, activated by preoperative radiation therapy (RT), doubled the rate of pathologic complete response after resection compared with preoperative RT alone in adult patients with locally advanced soft tissue sarcoma of the extremity or trunk wall (16.1% vs 7.9%, P = .045), and more patients achieved R0 resections (77.0% vs 64.0%, P = .042). These are the toxicity and health-related quality of life (HRQoL) results. Methods and Materials Act.In.Sarc randomized eligible patients 1:1 to either NBTXR3 (single intratumoral injection, volume equivalent to 10% of baseline tumor volume, at 53.3 g/L) activated by external-beam RT (arm A) or external-beam RT alone (arm B) (50 Gy in 25 fractions), followed by surgery in both arms. Here, we report the safety analyses in the all-treated population with a long-term follow-up of at least 2 years, and HRQoL in the intention-to-treat full analysis set. Results During the on-treatment period, serious adverse events (SAEs) of all grades related to NBTXR3 occurred in 10.1% (9/89) of patients (arm A), and SAEs related to RT occurred in 5.6% (5/89) (arm A) versus 5.6% (5/90) (arm B); postsurgery hospitalization owing to SAEs occurred in 15.7% (14/89) (arm A) versus 24.4% (22/90) (arm B). During the follow-up period, posttreatment SAEs (regardless of relationship) occurred in 13.5% (12/89) (arm A) versus 24.4% (22/90) (arm B). NBTXR3 did not negatively affect HRQoL; during the follow-up period, there was an improvement in most mean Toronto extremity salvage, EuroQoL 5-dimension (EQ-5D), EQ5D02-EQ visual analog scale, reintegration to normal living index, and musculoskeletal tumor rating scale scores. Conclusions NBTXR3 did not negatively affect safety or HRQoL. Long-term safety results reinforce the favorable benefit–risk ratio of NBTXR3 plus RT.

    وصف الملف: application/pdf

  8. 8
    دورية أكاديمية

    المصدر: urn:ISSN:0923-7534 ; urn:ISSN:1569-8041 ; Annals of Oncology, 31, 11, 1506-1517

    الوصف: Sarcomas are a heterogeneous group of malignancies with mesenchymal lineage differentiation. The discovery of neurotrophic tyrosine receptor kinase (NTRK) gene fusions as tissue-agnostic oncogenic drivers has led to new personalized therapies for a subset of patients with sarcoma in the form of tropomyosin receptor kinase (TRK) inhibitors. NTRK gene rearrangements and fusion transcripts can be detected with different molecular pathology techniques, while TRK protein expression can be demonstrated with immunohistochemistry. The rarity and diagnostic complexity of NTRK gene fusions raise a number of questions and challenges for clinicians. To address these challenges, the World Sarcoma Network convened two meetings of expert adult oncologists and pathologists and subsequently developed this article to provide practical guidance on the management of patients with sarcoma harboring NTRK gene fusions. We propose a diagnostic strategy that considers disease stage and histologic and molecular subtypes to facilitate routine testing for TRK expression and subsequent testing for NTRK gene fusions.

    وصف الملف: application/pdf

  9. 9
    دورية أكاديمية
  10. 10

    المساهمون: Casali, P.G., Abecassis, N., Bauer, S., Biagini, R., Bielack, S., Bonvalot, S., Boukovinas, I., Bovee, J.V.M.G., Brodowicz, T., Broto, J.M., Buonadonna, A., De Álava, E., Dei Tos, A.P., Del Muro, X.G., Dileo, P., Eriksson, M., Fedenko, A., Ferraresi, V., Ferrari, A., Ferrari, S., Frezza, A.M., Gasperoni, S., Gelderblom, H., Gil, T., Grignani, G., Gronchi, A., Haas, R.L., Hannu, A., Hassan, B., Hohenberger, P., Issels, R., Joensuu, H., Jones, R.L., Judson, I., Jutte, P., Kaal, S., Kasper, B., Kopeckova, K., Krákorová, D.A., Le Cesne, A., Lugowska, I., Merimsky, O., Montemurro, M., Pantaleo, M.A., Piana, R., Picci, P., Piperno-Neumann, S., Pousa, A.L., Reichardt, P., Robinson, M.H., Rutkowski, P., Safwat, A.A., Schöffski, P., Sleijfer, S., Stacchiotti, S., Sundby Hall, K., Unk, M., Van Coevorden, F., Van Der Graaf, W., Whelan, J., Wardelmann, E., Zaikova, O., Blay, J.Y., Man, Biomaterials and Microbes (MBM), Public Health Research (PHR), ​Basic and Translational Research and Imaging Methodology Development in Groningen (BRIDGE), Heikki Joensuu / Principal Investigator, Department of Oncology, Clinicum, HUS Comprehensive Cancer Center, [Casali, P. G.] Fdn IRCCS Ist Nazl Tumori, Milan, Italy, [Frezza, A. M.] Fdn IRCCS Ist Nazl Tumori, Milan, Italy, [Gronchi, A.] Fdn IRCCS Ist Nazl Tumori, Milan, Italy, [Casali, P. G.] Univ Milan, Milan, Italy, [Frezza, A. M.] Univ Milan, Milan, Italy, [Gronchi, A.] Univ Milan, Milan, Italy, [Abecassis, N.] EPE, Inst Portugues Oncol Lisboa Francisco Gentil, Lisbon, Portugal, [Bauer, S.] Univ Hosp Essen, Essen, Germany, [Biagini, R.] Regina Elena Inst Canc Res, IFO, Musculoskeletal Tissue Bank, Dept Oncol Orthoped, Rome, Italy, [Bielack, S.] Olga Hosp, Klinikum Stuttgart, Stuttgart, Germany, [Bonvalot, S.] Inst Curie, Paris, France, [Piperno-Neumann, S.] Inst Curie, Paris, France, [Boukovinas, I.] NORDIX, Athens, Greece, [Bovee, J. V. M. G.] Leiden Univ, Med Ctr, Dept Pathol, Leiden, Netherlands, [Brodowicz, T.] Med Univ Wien, Vienna Gen Hosp AKH, Vienna, Austria, [Broto, J. M.] Hosp Univ Virgen Rocio, CIBERONC, Seville, Spain, [De Alava, E.] Hosp Univ Virgen Rocio, CIBERONC, Seville, Spain, [Buonadonna, A.] Ctr Riferimento Oncol Aviano, Aviano, Italy, [Dei Tos, A. P.] Osped Reg Treviso S Maria Ca Foncello, Treviso, Italy, [Del Muro, X. G.] HUB, ICO Hosp, Integrated Unit, Barcelona, Spain, [Dileo, P.] Univ Coll London Hosp, Sarcoma Unit, London, England, [Eriksson, M.] Skane Univ Hosp Lund, Lund, Sweden, [Fedenko, A.] NN Blokhin Russian Canc Res Ctr, Moscow, Russia, [Ferraresi, V.] Inst Sci Hosp Care, Regina Elena Natl Canc Inst, IRCCS, Rome, Italy, [Ferrari, A.] Fdn IRCCS Ist Nazl Tumori, Pediat Oncol Unit, Milan, Italy, [Ferrari, S.] Ist Ortoped Rizzoli, Bologna, Italy, [Picci, P.] Ist Ortoped Rizzoli, Bologna, Italy, [Gasperoni, S.] Univ Careggi Firenze, Azienda Osped, Florence, Italy, [Gelderblom, H.] Leiden Univ, Med Ctr, Dept Med Oncol, Leiden, Netherlands, [Gil, T.] Inst Jules Bordet, Brussels, Belgium, [Grignani, G.] IRCCS, FPO, Candiolo Canc Inst, Candiolo, Italy, [Haas, R. L.] Netherlands Canc Inst, Dept Radiotherapy, Amsterdam, Netherlands, [Haas, R. L.] Leiden Univ, Med Ctr, Dept Radiotherapy, Leiden, Netherlands, [Hannu, A.] Turku Univ Hosp, Turun Yliopistollinen Keskussairaala, Turlu, Finland, [Hassan, B.] Oxford Univ Hosp NHS Fdn Trust, Oxford, England, [Hohenberger, P.] Mannheim Univ, Med Ctr, Mannheim, Germany, [Kasper, B.] Mannheim Univ, Med Ctr, Mannheim, Germany, [Issels, R.] Ludwig Maximilians Univ Munchen, Univ Hosp, Dept Med 3, Munich, Germany, [Joensuu, H.] Univ Helsinki, Cent Hosp, HUCH, Helsinki, Finland, [Jones, R. L.] Royal Marsden Hosp, London, England, [Van der Graaf, W.] Royal Marsden Hosp, London, England, [Judson, I.] Inst Canc Res, London, England, [Jutte, P.] Univ Med Ctr Groningen, Groningen, Netherlands, [Kaal, S.] Radboud Univ Nijmegen, Med Ctr, Nijmegen, Netherlands, [Kopeckova, K.] Univ Hosp Motol, Prague, Czech Republic, [Krakorova, D. A.] Masaryk Mem Canc Inst, Brno, Czech Republic, [Le Cesne, A.] Gustave Roussy Canc Campus, Villejuif, France, [Lugowska, I.] Marie Sklodowska Curie Inst, Oncol Ctr, Warsaw, Poland, [Rutkowski, P.] Marie Sklodowska Curie Inst, Oncol Ctr, Warsaw, Poland, [Merimsky, O.] Tel Aviv Sourasky Med Ctr Ichilov, Tel Aviv, Israel, [Montemurro, M.] Univ Hosp Lausanne, Med Oncol, Lausanne, Switzerland, [Pantaleo, M. A.] Univ Bologna, Policlin S Orsola Malpighi, Azienda Osped, Bologna, Italy, [Piana, R.] Univ Cita Salute & Sci Torino, Azienda Osped, Turin, Italy, [Pousa, A. L.] Hosp Santa Creu & Sant Pau, Fundacio Gestio Sanitaria, Barcelona, Spain, [Reichardt, P.] Helios Klinikum Berlin Buch, Berlin, Germany, [Robinson, M. H.] Weston Pk Hosp NHS Trust, Dept Clin Oncol, YCRC, Sheffield, S Yorkshire, England, [Safwat, A. A.] Aarhus Univ Hosp, Aarhus, Denmark, [Schoffski, P.] Leuven Canc Inst, Leuven, Belgium, [Sleijfer, S.] Erasmus MC Canc Inst, Dept Med Oncol, Rotterdam, Netherlands, [Stacchiotti, S.] Ist Nazl Tumori, Fdn Ist Ricovero & Cura Carattere Sci, Milan, Italy, [Hall, K. Sundby] Norwegian Radium Hosp, Oslo Univ Hosp, Dept Oncol, Oslo, Norway, [Unk, M.] Inst Oncol Ljubljana, Ljubljana, Slovenia, [Van Coevorden, F.] Netherlands Canc Inst Antoni van Leeuwenhoek, Amsterdam, Netherlands, [Whelan, J.] Univ Coll Hosp, London, England, [Wardelmann, E.] Univ Klinikum Munster, Gerhard Domagk Inst Pathol, Munster, Germany, [Zaikova, O.] Norwegian Radium Hosp, Oslo Univ Hosp, Oslo, Norway, [Blay, J. Y.] Ctr Leon Bernard, Lyon, France, [Blay, J. Y.] UCBL1, Lyon, France, Amgen Dompe, AROG Bayer, Blueprint Medicines, Eli Lilly, Daiichi Sankyo Pharma, Epizyme, GlaxoSmithKline, Novartis, Pfizer, PharmaMar, Incyte, Lilly, Bayer, Janssen-Cilag, Eisai, Loxo Oncology, Nanobiotix, Bristol-Myers Squibb, Merck Sharp Dohme, Roche, Amgen, EuroSarc, CoBioRes, Exelixis, Plexxikon, Sixth Element Capital, Adaptaimmune, Amcure, AstraZeneca, Boehringer Ingelheim, Cristal Therapeutics, Daichii Sankyo Pharma, Genzyme, Ipsen, Medpace, Nektar, Philogen, Piqur Therapeutics, Swedish Orphan Biovitrium, Advenchen, Epizyme Inc., PharmarMar, Novartis Oncology, Milestone, Menarini, Medical Oncology

    المصدر: Annals of Oncology, 29, iv68-iv78
    Annals of Oncology, 29, Supplement_4, pp. iv68-iv78
    Annals of Oncology, 29, 68-78
    ANNALS OF ONCOLOGY
    r-IIB SANT PAU. Repositorio Institucional de Producción Científica del Instituto de Investigación Biomédica Sant Pau
    instname
    Annals of Oncology, 29, 68-78. Oxford University Press
    Annals of Oncology, 29, 68-78. Elsevier Ltd.

    الوصف: Gastrointestinal stromal tumours (GISTs) are rare tumours, with an estimated unadjusted incidence of around 1/100 000/year. This only covers clinically relevant GISTs, since, if investigated, amuch higher number of lesions ≤ 1 cmin diameter (microGISTs) can be found at histopathological examination of stomach tissue in middle-aged and elderly individuals. There is a slight prevalence in males. The median age is around 60–65 years, with a wide range. Occurrence in children is very rare. Paediatric GIST represents a clinically and molecularly distinct subset, marked by female predominance, absence of KIT/platelet- derived growth factor alpha (PDGFRA) mutations, frequent mutations or silencing of the four genes that encode the subunits of the succinate dehydrogenase (SDH) enzyme complex, gastric multicentric location and possible lymph node metastases. Some syndromes are linked to GISTs: • The Carney triad syndrome, marked by gastric GISTs, paraganglioma and pulmonary chondromas (these may occur at different ages); • Carney–Stratakis syndrome, marked by a dyad of GIST and paraganglioma; and • Neurofibromatosis type 1(NF1), possibly leading to wild-type (WT), often multicentric GIST, predominantly located in the small bowel Families with germline autosomal dominant mutations of KIT are an extremely rare finding, presenting with multiple GISTs at an early age, possibly along with other associated features such as pigmented skin macules, urticaria pigmentosa and diffuse hyperplasia of the interstitial cells of Cajal in the gut wall.

    وصف الملف: ELETTRONICO