Clinical characteristics and clinical evolution of a large cohort of pediatric patients with primary central nervous system (cns) tumors and tropomyosin receptor kinase (trk) fusion.

التفاصيل البيبلوغرافية
العنوان:	Clinical characteristics and clinical evolution of a large cohort of pediatric patients with primary central nervous system (cns) tumors and tropomyosin receptor kinase (trk) fusion.
المؤلفون:	Lamoureux A.-A., Fisher M., Lemelle L., Pfaff E., Kramm C., De Wilde B., Kazanowska B., Hutter C., Pfister S.M., Sturm D., Jones D., Orbach D., Pierron G., Raskin S., Drilon A., Diamond E., Harada G., Zapotocky M., Ellezam B., Weil A.G., Venne D., Barritault M., Leblond P., Coltin H., Hammad R., Tabori U., Hawkins C., Hansford J.R., Meyran D., Erker C., McFadden K., Sato M., Gottardo N.G., Dholaria H., Noroxe D.S., Goto H., Ziegler D.S., Lin F.Y., Parsons D.W., Lindsay H., Wong T.-T., Liu Y.-L., Wu K.-S., Franson A.F., Hwang E., Aguilar-Bonilla A., Cheng S., Cacciotti C., Massimino M., Schiavello E., Wood P., Hoffman L.M., Cappellano A., Lassaletta A., Van Damme A., Llort A., Gerber N.U., Ceruso M.S., Bendel A.E., Skrypek M., Hamideh D., Mushtaq N., Walter A., Jabado N., Alsahlawi A., Farmer J.-P., Abadi C.C., Mueller S., Mazewski C., Aguilera D., Robison N., O'Halloran K., Abbou S., Berlanga P., Geoerger B., Ora I., Moertel C.L., Razis E.D., Vernadou A., Doz F., Laetsch T.W., Perreault S.
بيانات النشر:	Oxford University Press Netherlands 2022-07-23
نوع الوثيقة:	Electronic Resource
مستخلص:	BACKGROUND: TRK fusions are detected in less than 3% of CNS tumors. Given their rarity, there are limited data on the clinical course of these patients. METHOD(S): We contacted 166 oncology centers worldwide to retrieve data on patients with TRK fusion-driven CNS tumors. Data extracted included demographics, histopathology, NTRK gene fusion, treatment modalities and outcomes. Patients less than 18 years of age at diagnosis were included in this analysis. RESULT(S): Seventy-three pediatric patients with TRK fusion-driven primary CNS tumors were identified. Median age at diagnosis was 2.4 years (range 0.0-17.8) and 60.2 % were male. NTRK2 gene fusions were found in 37 patients (50.7%), NTRK1 and NTRK3 aberrations were detected in 19 (26.0%) and 17 (23.3%), respectively. Tumor types included 38 high-grade gliomas (HGG; 52.1%), 20 low-grade gliomas (LGG; 27.4%), 4 embryonal tumors (5.5%) and 11 others (15.1%). Median follow-up was 46.5 months (range 3-226). During the course of their disease, a total of 62 (84.9%) patients underwent surgery with a treatment intent, 50 (68.5%) patients received chemotherapy, 35 (47.9%) patients received radiation therapy, while 34 (46.6%) patients received NTRK inhibitors (3 as first line treatment). Twenty-four (32.9%) had no progression including 9 LGG (45%) and 9 HGG (23.6%). At last follow-up, only one (5.6%-18 evaluable) patient with LGG died compared to 11 with HGG (35.5%-31 evaluable). For LGG the median progression-free survival (PFS) after the first line of treatment was 17 months (95% CI: 0.0-35.5) and median overall survival (OS) was not reached. For patients with HGG the median PFS was 30 months (95% CI: 11.9-48.1) and median OS was 182 months (95% CI 20.2-343.8). CONCLUSION(S): We report the largest cohort of pediatric patients with TRK fusion-driven primary CNS tumors. These results will help us to better understand clinical evolution and compare outcomes with ongoing clinical trials.
مصطلحات الفهرس:	Conference Abstract
URL:	Neuro-Oncology
الاتاحة:	Open access content. Open access content
Other Numbers:	AUSHL oai:repository.monashhealth.org:1/48255 Neuro-Oncology. Conference: 20th International Symposium on Pediatric Neuro-Oncology, ISPNO 2022. Hamburg Germany. 24(Supplement 1) (pp i61-i62), 2022. Date of Publication: June 2022. https://repository.monashhealth.org/monashhealthjspui/handle/1/48255 (Lamoureux, Ellezam, Weil, Venne, Perreault) CHU Sainte-Justine, Montreal, Canada (Fisher, Laetsch) Children's Hospital of Philadelphia, Philadelphia, United States (Lemelle, Orbach, Pierron, Doz) Institut Curie, Paris, France (Pfaff, Pfister, Sturm, Jones) Hopp Children's Cancer Center Heidelberg (KiTZ), German Cancer Research Center (DKFZ), German Cancer Consortium (DKTK), Heidelberg, Germany (Kramm) University Medical Center Gottingen, Gottingen, Germany (De Wilde) Ghent University Hospital, Ghent, Belgium (Kazanowska) Department of Pediatric Hematology/Oncology and BMT, Wroclaw Medical University, Wroclaw, Poland (Hutter) St. Anna Children's Hospital, Depart-ment of Pediatrics, Medical University of Vienna, and St. Anna Children's Cancer Research Institute (CCRI), Viennes, Austria (Raskin, Diamond, Harada) Cincinnati Children's Hospital Medical Center, Cincinnati, United States (Drilon) Memorial Sloan Kettering Cancer Center, New York City, United States (Zapotocky) Department of Paediatric Haematology and Oncology, Second Faculty of Medicine, Charles University and University Hospital Motol, Prague, Czechia (Barritault) Hospices Civils de Lyon, Lyon, France (Leblond) Institut d'Hematologie et d'Oncologie Pediatrique, Pluridisciplinar Research in pediatric Oncology for Perspectives in Evaluation Care and Therapy (PROSPECT), Centre Leon Berard, Lyon, France (Coltin, Hammad, Tabori, Hawkins) Hospital for Sick Children, Toronto, Canada (Hammad) Hematology department, Faculty of Medicine, King Abdulaziz University, Jeddah, Saudi Arabia (Hansford, Meyran) Royal Children's Hospital; Murdoch Children's Research Institute, Department of Pediatrics, University of Melbourne, Melbourne, Australia (Erker, McFadden) IWK Health Centre, Halifax, Canada (Sato) University of Iowa Stead Family Children's Hospital, Iowa City, United States (Gottardo, Dholaria) Perth Children's Hospital; Brain Tumour Research Programme, Telethon Kids Institute; Paediatrics, University of Western Au (Wood) Monash Children's Hospital, Monash University, Hudson Institute of Medical Research, Clayton, Australia 1346232076
المصدر المساهم:	MONASH HEALTH LIBRS From OAIster®, provided by the OCLC Cooperative.
رقم الانضمام:	edsoai.on1346232076
قاعدة البيانات:	OAIster

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