Given limited evidence, optimal management remains undefined however surgical cytoreduction continues to be the cornerstone of management across reported cases with adjuvant treatment individualised. Certain fertility preservation strategies are concurrently feasible within a multidisciplinary framework and should be considered given reported cases are predominantly seen in younger females.

1 day ago

Journal

PGR (Progesterone receptor) • MUC16 (Mucin 16, Cell Surface Associated)

PNOC027: Individualized Treatment Plan in Children and Young Adults With Relapsed Medulloblastoma and Ependymoma (clinicaltrials.gov)

P=N/A, N=74, Recruiting, University of California, San Francisco | Suspended --> Recruiting

7 days ago

Enrollment open

BrainChild-03: Study of B7-H3-Specific CAR T Cell Locoregional Immunotherapy for Diffuse Intrinsic Pontine Glioma/Diffuse Midline Glioma and Recurrent or Refractory Pediatric Central Nervous System Tumors (clinicaltrials.gov)

P1, N=90, Recruiting, Seattle Children's Hospital | Trial completion date: May 2041 --> May 2042 | Trial primary completion date: May 2026 --> May 2027

8 days ago

Trial completion date • Trial primary completion date

SCRI-CARB7H3(s)

Lutathera for Treatment of Recurrent or Progressive High-Grade CNS Tumors (clinicaltrials.gov)

P1/2, N=65, Recruiting, Nationwide Children's Hospital | Trial completion date: Nov 2027 --> Nov 2033 | Trial primary completion date: Nov 2027 --> Nov 2028

9 days ago

Trial completion date • Trial primary completion date

Lutathera (lutetium Lu 177 dotatate)

PNOC027: Individualized Treatment Plan in Children and Young Adults With Relapsed Medulloblastoma and Ependymoma (clinicaltrials.gov)

P=N/A, N=74, Suspended, University of California, San Francisco | Recruiting --> Suspended

9 days ago

Trial suspension

14d

Role of Surrogate Immunohistochemistry Markers in CNS Tumors in the Era of Molecular Diagnostics With Recent Updates. (PubMed, Adv Anat Pathol)

The review emphasizes that when interpreted alongside histomorphology and conventional markers, surrogate immunohistochemistry can significantly reduce reliance on molecular testing, expedite diagnosis, and improve accessibility of precision diagnostics. Standardization, validation, and awareness of pitfalls remain essential to maximizing their clinical utility in neuropathology practice.

14 days ago

Journal

BRAF (B-raf proto-oncogene) • ALK (Anaplastic lymphoma kinase) • SMARCA4 (SWI/SNF related, matrix associated, actin dependent regulator of chromatin, subfamily A, member 4) • CTNNB1 (Catenin (cadherin-associated protein), beta 1) • ATRX (ATRX Chromatin Remodeler) • SMARCB1 (SWI/SNF Related, Matrix Associated, Actin Dependent Regulator Of Chromatin, Subfamily B, Member 1) • BCOR (BCL6 Corepressor) • YAP1 (Yes associated protein 1) • VIM (Vimentin) • STAT6 (Signal transducer and activator of transcription 6) • L1CAM (L1 cell adhesion molecule) • DUX4 (Double Homeobox 4) • FOXR2 (Forkhead Box R2) • GAB1 (GRB2 Associated Binding Protein 1)

TP53 mutation • BRAF V600E • BRAF V600 • IDH mutation + BRAF V600E

15d

Intraocular Ependymoma in a Child - Case report. (PubMed, Retin Cases Brief Rep)

Although extremely rare, intraocular ependymoma should be included as part of the differential diagnosis of retinal and ciliary body tumors in childhood.

15 days ago

Journal

L1CAM (L1 cell adhesion molecule) • GFAP (Glial Fibrillary Acidic Protein) • OLIG2 (Oligodendrocyte Transcription Factor 2) • RELA (RELA Proto-Oncogene) • ZFTA (Zinc Finger Translocation Associated)

17d

Genome-wide profiling of lncRNAs in pediatric intracranial ependymomas identifies H19 as a novel pathogenic driver. (PubMed, Neurooncol Adv)

Finally, public data showed that H19 is more abundant in the EPN PF subgroup A, and that methylation of its imprinting control region (ICR) correlates strongly with better prognosis in EPN PF subgroup B (PFB). These findings suggest that H19 plays an oncogenic role in EPN and that the methylation status of its ICR may serve as a prognostic biomarker in PFB.

17 days ago

Journal

IGF2 (Insulin-like growth factor 2)

21d

Molecular Biology and Phase 1 Study of GM-CSF and Intrathecal Trastuzumab in Children with Recurrent Posterior Fossa Ependymoma. (PubMed, Neuro Oncol)

IT trastuzumab in combination with GM-CSF is safe in recurrent pediatric PF EPN. A larger phase 2 study that includes both recurrent PFA and RELA-ST EPN is needed to determine the long-term efficacy of this immunotherapy strategy.

21 days ago

P1 data • Journal • IO biomarker

HER-2 (Human epidermal growth factor receptor 2) • CSF2 (Colony stimulating factor 2) • RELA (RELA Proto-Oncogene)

Herceptin (trastuzumab)

25d

Cauda equina neuroendocrine tumors: An 11-Year retrospective report on clinicopathological characteristics and postoperative recurrence. (PubMed, Neurochirurgie)

CENETS are rare and often misdiagnosed. Serpentine dilated vessel identification at the tumor's cephalad part during surgery may be indicative, and gross total resection be curative.

25 days ago

Retrospective data • Journal

GATA3 (GATA binding protein 3) • SYP (Synaptophysin) • GFAP (Glial Fibrillary Acidic Protein) • OLIG2 (Oligodendrocyte Transcription Factor 2)