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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; [...]

ETMR-22. TITLE: DEFINING THE CLINICAL AND PROGNOSTIC LANDSCAPE OF EMBRYONAL TUMORS WITH MULTI-LAYERED ROSETTES (ETMRs), A RARE BRAIN TUMOR REGISTRY (RBTC) STUDY

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  • Media type: E-Article
  • Title: ETMR-22. TITLE: DEFINING THE CLINICAL AND PROGNOSTIC LANDSCAPE OF EMBRYONAL TUMORS WITH MULTI-LAYERED ROSETTES (ETMRs), A RARE BRAIN TUMOR REGISTRY (RBTC) STUDY
  • Contributor: 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; [...]
  • imprint: Oxford University Press (OUP), 2020
  • Published in: Neuro-Oncology, 22 (2020) Supplement_3, Seite iii327-iii328
  • Language: English
  • DOI: 10.1093/neuonc/noaa222.225
  • ISSN: 1522-8517; 1523-5866
  • Keywords: Cancer Research ; Neurology (clinical) ; Oncology
  • Origination:
  • Footnote:
  • Description: <jats:title>Abstract</jats:title> <jats:p>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&amp;lt;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.</jats:p>
  • Access State: Open Access