Ependymoma

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.

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.

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.

In vivo lineage tracing studies identified neoplastic clones that aggressively dominated tumour growth and established the entire EPN cellular hierarchy. These findings identify developmental epigenomic states that are critical for fusion-oncoprotein-driven transformation and show how these states continue to shape tumour progression.

P2, N=39, Not yet recruiting, Clinica Universidad de Navarra, Universidad de Navarra | N=20 --> 39

Several significant intronic variants were harbored in genes associated with cancer and neurological disease. Future studies are needed to investigate the role of these age-specific alterations in ependymoma pathogenesis.

P=N/A, N=74, Recruiting, University of California, San Francisco | Trial primary completion date: Dec 2025 --> Dec 2027

P2, N=30, Recruiting, University of California, San Francisco | Not yet recruiting --> Recruiting

This study illustrates the underestimated high frequency of mosaic NF2-related schwannomatosis in patients harboring multi-nodular peripheral nerve schwannomas. As multi-nodular schwannomas are associated with higher surgical morbidity, the pivotal role of the NF2 gene in their tumorigenesis opens perspectives for Schwannomatosis research.

P1/2, N=20, Not yet recruiting, Clinica Universidad De Navarra

Most of these tumors are classified in the "no specific molecular profile" subgroup and have high rates of nuclear beta-catenin/CTNNB1 alterations. Awareness of these morphologic and immunohistochemical associations may help avoid misclassification as sex cord-stromal or other neoplasms.

P1/2, N=68, Not yet recruiting, Washington University School of Medicine | Trial primary completion date: Mar 2031 --> Jun 2031