Malignant Ependymoblastoma Mimicking a Benign Pilocytic Astrocytoma
Pham Minh Thong1, Nguyen Minh Duc1,2,3
1Department of Radiology, Hanoi Medical University, Ha Noi 100000, Vietnam.
Insights
Ependymoblastoma, a rare and aggressive brain tumor in children, is often misdiagnosed due to non-specific imaging features. This case highlights a fourth-ventricular ependymoblastoma misidentified as pilocytic astrocytoma despite advanced MRI.
Area of Science:
- Pediatric Neuro-oncology
- Central Nervous System Tumors
- Diagnostic Imaging
Background:
- Ependymoblastoma is a rare, highly malignant pediatric brain tumor (WHO Grade IV).
- It is a subtype of primitive neuroectodermal tumors, often arising in the supratentorial region and near the ventricular system.
- Histopathology shows small, blue cells with rosettes and high mitotic activity.
Observation:
- Clinical and imaging features of ependymoblastomas are often non-specific.
- This can lead to misdiagnosis as other brain neoplasms.
- A case of fourth-ventricular ependymoblastoma was initially misdiagnosed as pilocytic astrocytoma.
Findings:
- The misdiagnosis occurred despite the use of advanced magnetic resonance imaging (MRI) protocols.
- This underscores the diagnostic challenges posed by ependymoblastoma.
- Histopathological confirmation is crucial for accurate diagnosis.
Implications:
- Improved diagnostic criteria and awareness are needed for ependymoblastoma.
- Early and accurate diagnosis is critical for appropriate treatment and improved outcomes in pediatric brain tumors.
- This case emphasizes the importance of considering rare diagnoses even with advanced imaging.
Abstract:
Ependymoblastoma is an uncommon, exceedingly malignant brain neoplasm that adversely influences children's quality of life. Ependymoblastoma represents a subtype of primitive neuroectodermal tumors, categorized as grade IV, according to the 2007 World Health Organization (WHO) classification of central nervous system tumors. Ependymoblastomas are often located in the supratentorial zone and often associated with the ventricular system. Histopathological sections of the tumor revealed uniform, primitive, small blue cells, with multi-layered rosettes, accompanied by abundant mitoses. The clinical and imaging features of ependymoblastomas are not specific, which can result in misdiagnosis as other brain neoplasms. In this paper, we described the identification of a fourth-ventricular ependymoblastoma that was misdiagnosed as pilocytic astrocytoma, despite the utilization of advanced magnetic resonance imaging (MRI) protocols.
