دورية أكاديمية
ETMR-22. TITLE: DEFINING THE CLINICAL AND PROGNOSTIC LANDSCAPE OF EMBRYONAL TUMORS WITH MULTI-LAYERED ROSETTES (ETMRs), A RARE BRAIN TUMOR REGISTRY (RBTC) STUDY
| العنوان: | ETMR-22. TITLE: DEFINING THE CLINICAL AND PROGNOSTIC LANDSCAPE OF EMBRYONAL TUMORS WITH MULTI-LAYERED ROSETTES (ETMRs), A RARE BRAIN TUMOR REGISTRY (RBTC) STUDY |
|---|---|
| المؤلفون: | Khan, Sara, Solano-Paez, Palma, Suwal, Tannu, Al-Karmi, Salma, Lu, Mei, Ho, Ben, Fouladi, Maryam, Leary, Sarah, Levy, Jean M Mulcahy, Lassaletta, Alvaro, Rivas, Eloy, Reddy, Alyssa, Gillespie, G Yancey, Gupta, Nalin, Yalon-Oren, Michal, Amariglio, Laura, Nakamura, Hideo, Wu, Kuo-Sheng, Wong, Tai-Tong, Ra, Young-Shin, Spina, Milena La, Emanuele, Policlinico Vittorio, Massimi, Luca, Buccoliero, Anna Maria, Hansford, Jordan R, Grundy, Richard G, Adamek, Dariusz, Fangusaro, Jason, Scharnhorst, David, Johnston, Donna, Lafay-Cousin, Lucie, Camelo-Piragua, Sandra, Kabbara, Nabil, Gajjar, Amar, Boutarbouch, Mahjouba, da Costa, Maria Joao Gil, Hanson, Derek, Wood, Paul, Al-Hussaini, Maysa, Amayiri, Nisreen, Wang, Yin, Catchpoole, Daniel, Michaud, Jean, Bendel, Anne E, Ellezam, Benjamin, Gerber, Nicholas, Plant, Ashley, Jeffery, Rubens, Dunham, Christopher, Moertel, Christopher |
| المصدر: | Neuro-Oncology ; volume 22, issue Supplement_3, page iii327-iii328 ; ISSN 1522-8517 1523-5866 |
| بيانات النشر: | Oxford University Press (OUP) |
| سنة النشر: | 2020 |
| مصطلحات موضوعية: | Cancer Research, Neurology (clinical), Oncology |
| الوصف: | ETMR, an aggressive disease characterised by C19MC alterations, were previously categorised as various histologic diagnoses. The clinical spectrum and impact of conventional multi-modal therapy on this new WHO diagnostic category remains poorly understood as a majority of ~200 cases reported to date lack molecular confirmation. We undertook comprehensive clinico-pathologic studies of a large molecularly confirmed cohort to improve disease recognition and treatment approaches. Amongst 623 CNS-PNETs patients enrolled in the RBTC registry, 159 primary ETMRs were confirmed based on a combination of FISH (125), methylation analysis (88), SNP and RNAseq (32) analyses; 91% had C19MC amplification/gains/fusions, 9% lacked C19MC alterations but had global methylation features of ETMR NOS. ETMRs arose in young patients (median age 26 months) predominantly as localized disease (M0-72%, M2-3 -18%) at multiple locations including cerebrum (60%) cerebellum (18%), midline structures (6%); notably 10% were brainstem primaries mimicking DIPG. Uni-and multivariate analyses of clinical and treatment details of curative regimens available for 110 patients identified metastatic disease (p=0.002), brainstem locations(p=0.005), extent of surgery, receipt of multi-modal therapy including high dose chemotherapy and radiation (P<0.001) as significant treatment prognosticators, while C19MC status, age and gender were non-significant risk factors. Analyses of events in all patients showed respective EFS at 3 and 12 months of 84%(95%CI:77–91) and 37%(95%CI:20–41) and 4yr OS of 27%(95%CI:18–37) indicating despite intensified therapies ETMR is a rapidly progressive and fatal disease. Our comprehensive data on the largest cohort of molecularly-confirmed ETMRs provides a critical framework to guide current clinical management and development of clinical trials. |
| نوع الوثيقة: | article in journal/newspaper |
| اللغة: | English |
| DOI: | 10.1093/neuonc/noaa222.225 |
| الإتاحة: | https://doi.org/10.1093/neuonc/noaa222.225Test http://academic.oup.com/neuro-oncology/article-pdf/22/Supplement_3/iii327/34686782/noaa222.225.pdfTest |
| حقوق: | http://creativecommons.org/licenses/by-nc/4.0Test/ |
| رقم الانضمام: | edsbas.B72D202E |
| قاعدة البيانات: | BASE |
| DOI: | 10.1093/neuonc/noaa222.225 |
|---|