Rare Embryonal Tumors of the Central Nervous System: International Registry

Summary

Central nervous system (CNS) tumors are the most common solid malignancies among children. Although some types of CNS tumors like medulloblastomas and low-grade gliomas are widespread and well-studied, there is a huge number of rare diseases that need further research. This international registry aims to establish a large multicenter database of pediatric and young adult patients with rare embryonal tumors of the central nervous system and describe the clinical presentations, diagnostics, treatment regimens, and outcomes. Embryonal tumors with multilayered rosettes (ETMR), FOXR2-activated CNS neuroblastoma, cribriform neuroepithelial tumor, and CNS tumor with BCOR internal tandem duplication are extremely rare embryonal tumors some of which were first described in the last edition of the World Health Organization (WHO) Classification of Tumors of the Central Nervous System. Objectives of the registry are 1) to evaluate prognostic factors, 2) to identify diagnostic and treatment gaps, 3) to investigate the characteristics and outcome of the disease with different treatment regimens, and 4) to generate data-based prospective diagnostic and treatment recommendations.

Eligibility

Inclusion Criteria:

* Patients diagnosed with rare embryonal tumors of CNS since 01.01.2010:

  * ETMR (including embryonal tumor with abundant neuropil and true rosettes (ETANTR), ependymoblastoma (EBL) and medulloepithelioma (MEPL) which were previously classified as CNS-PNETs)
  * FOXR2-activated CNS neuroblastoma
  * cribriform neuroepithelial tumor
  * CNS tumor with BCOR internal tandem duplication
  * all patients diagnosed with neuroblastoma and ganglioneuroblastoma with no molecular genetic tests available
* Patients ≤ 25 years of age
* Signed informed consent form for prospective patients ≥ 18 years of age
* Signed parental permission and child assent forms for prospective patients \< 18 years of age

Exclusion Criteria:

• CNS metastases of extracranial embryonal tumors

Conditions2

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