Clinical phenotypes and prognostic features of embryonal tumours with multi-layered rosettes: a Rare Brain Tumor Registry study

Clinical phenotypes and prognostic features of ETMRs (Embryonal Tumor with Multi-layered Rosettes) a new CNS tumor entity: A Rare Brain Tumor Registry study

10.1101/2020.08.12.20171801 ◽

2020 ◽

Author(s):

Annie Huang

Keyword(s):

Brain Tumor ◽

High Dose Chemotherapy ◽

Treatment Modalities ◽

Patient Specific ◽

High Dose ◽

Total Resection ◽

Prognostic Features ◽

Clinical Phenotypes ◽

Diagnostic Practices ◽

Conventional Dose

Background ETMRs are a newly recognized rare paediatric brain tumor with alterations of the C19MC microRNA locus. Due to varied diagnostic practices and limited clinical data, disease features and determinants of outcome are poorly defined. We performed an integrated clinico-pathologic and molecular analyses of 159 primary ETMRs to define clinical phenotypes, identify predictors of survival and critical treatment modalities for this orphan disease. Methods Primary ETMR patients were identified from the Rare Brain Tumor Consortium (rarebraintumorconsortium.ca) global registry using histopathologic and molecular assays. Event-Free (EFS) and Overall Survival (OS) for 108 patients treated with curative multi-modal regimens were determined using Cox proportional hazard and log rank analyses. Findings ETMRs were predominantly non-metastatic (73%) tumors arising from multiple sites; 55% were cerebral tumors, 45% arose at sites characteristic of other brain tumors. Hallmark C19MC alterations were seen in 91%; 9% were ETMR-NOS. Survival and hazard analyses showed a 6 month median EFS and 2-4yr OS of 27-29% with metastatic disease (HR=0.44, 95% CI 0.26-0.74; p=0.002) and brainstem location (HR=0.40, 95% CI 0.021-0.75; p=0.005) correlating with adverse OS. Gross total resection (GTR: HR=0.38, 95% CI 0.21-0.68; p=0.001), high dose chemotherapy (HDC: HR=0.55, 95% CI 0.31-0.97; p=0.04) and radiation (RT: HR=0.32, 95% CI 0.16-0.60; p=<0.001) correlated with improved EFS and OS in multi-variable analyses. EFS and OS for patients treated with only conventional dose chemotherapy (CC) was 0% and respectively 37%+/-14% and 32%+/-13% for patients treated with HDC. Patients with GTR or sub-total resection (STR) treated with HDC and RT had superior EFS (GTR 73%+/-14%, p=0.018; STR 67%+/-19% p=0.009) and OS (GTR 66%+/-17%, p=0.05; STR 67%+/-16%, p=0.005). Amongst 21 long-term survivors (OS 24-202 months); 38%, 24% and 24% respectively received craniospinal, focal or no RT. Interpretation: Prompt molecular diagnosis and post-surgical treatment with multi-modal therapy tailored to patient-specific risk features improves ETMR survival.

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Effect of adjuvant radiotherapy after subtotal resection for WHO grade I meningioma: a propensity score matching analysis of the Brain Tumor Registry of Japan

Journal of Neuro-Oncology ◽

10.1007/s11060-021-03775-x ◽

2021 ◽

Author(s):

Soichi Oya ◽

Fusao Ikawa ◽

Nao Ichihara ◽

Masahiko Wanibuchi ◽

Yukinori Akiyama ◽

...

Keyword(s):

Brain Tumor ◽

Propensity Score ◽

Propensity Score Matching ◽

Adjuvant Radiotherapy ◽

Subtotal Resection ◽

Who Grade ◽

Tumor Registry ◽

Propensity Score Matching Analysis ◽

The Brain

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Construction of the National Brain Tumor Registry of China for better management and more efficient use of data: a protocol

BMJ Open ◽

10.1136/bmjopen-2020-040055 ◽

2021 ◽

Vol 11 (1) ◽

pp. e040055

Author(s):

Liwei Zhang ◽

Wang Jia ◽

Nan Ji ◽

Deling Li ◽

Dan Xiao ◽

...

Keyword(s):

Brain Tumor ◽

Clinical Research ◽

Brain Tumour ◽

Brain Tumours ◽

Benefit Sharing ◽

Data Quality Control ◽

Clinical Trial Registry ◽

Discharge Data ◽

Tumor Registry ◽

Depth Analysis

IntroductionBrain tumours encompass a complex group of intracranial tumours that mostly affect young adults and children, with a high incidence rate and poor prognosis. It remains impossible to systematically collect data on patients with brain tumours in China and difficult to perform in-depth analysis on the status of brain tumours, medical outcomes or other important medical issues through a multicentre clinical study. This study describes the first nation-wide data platform including the entire spectrum of brain tumour entities, which will allow better management and more efficient application of patient data in China.Methods and analysisThe National Brain Tumor Registry of China (NBTRC) is a registry of real-word clinical data on brain tumours. It is established and managed by the China National Clinical Research Center for Neurological Diseases and administered by its scientific and executive committees. The 54 participating hospitals of the NBTRC are located in 27 provinces/municipalities, performing more than 40 000 brain tumour surgeries per year. The data consist of in-hospital medical records, images and follow-up information after discharge. Data can be uploaded in three ways: the web portal, remote physical servers and offline software. The data quality control scheme is seven-dimensional. Each participating hospital could focus on a single pathology subtype and public subtypes of brain tumour for which they expect to conduct related multicentre clinical research. The standardised workflow to conduct clinical research is based on the benefit-sharing mechanism. Data collection will be conducted continuously from 1 February 2019 to 31 January 2024.Ethics and disseminationInformed consent will be obtained from all participants. Consent for the adolescents’ participation will be also obtained from their guardians via written consent. The results will be published in professional journals, in both Chinese and English.Trial registration numberChinese Clinical Trial Registry (ChiCTR1900021096).

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