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Malignant Transformation of Grade II Ependymoma in a 2-Year-Old Child: Case Report
Şule Sarıkafa1, Suat Erol Çelik1, Enver Yarikkaya2
1Department of Neurosurgery, Okmeydanı Training and Research Hospital, Istanbul, Turkey.
Abstract:
Ependymomas are central nervous system neoplasms that account for a third of all posterior fossa tumors in children. The most common location for infratentorial ependymoma is within the fourth ventricle. We present a case report of malignant transformation of an infratentorial grade II ependymoma in a 2-year-old child who presented with vomiting and visual disturbance. An infratentorial brain tumor in the left cerebellar area was totally removed, and the initial pathologic diagnosis was grade II ependymoma. The tumor recurred aggressively 1 year later; subtotal removal and adjuvant chemotherapy were performed. After a second operation, a histopathologic study was performed. The second specimen was defined as a grade III anaplastic ependymoma. Transformation to grade III anaplastic ependymoma is possible for a grade II ependymoma but very rare. The diagnosis of the anaplastic variant of intracranial ependymomas is difficult. Surgical treatment remains the mainstay of the treatment for all cases. Ependymomas in young infants have a worse prognosis than older children, so we need individual clinical evaluation and close follow-up of such cases. This article highlights the requirement of a close follow-up for grade II ependymomas for anaplastic transformation.
Insights
Malignant transformation of a grade II ependymoma to anaplastic ependymoma is rare but possible. Close follow-up of pediatric ependymoma cases is crucial for early detection of aggressive changes.
Area of Science:
- Pediatric neuro-oncology
- Central nervous system neoplasms
- Ependymoma research
Background:
- Ependymomas represent one-third of pediatric posterior fossa tumors.
- Infratentorial ependymomas most commonly occur in the fourth ventricle.
- Early diagnosis and treatment are critical for pediatric brain tumors.
Observation:
- A case report details a 2-year-old child with an infratentorial grade II ependymoma.
- The tumor recurred aggressively after initial treatment, progressing to grade III anaplastic ependymoma.
- Histopathologic analysis confirmed the malignant transformation.
Findings:
- Grade II ependymomas can undergo malignant transformation to grade III anaplastic ependymomas, though this is exceptionally rare.
- Diagnosis of the anaplastic variant of intracranial ependymomas presents diagnostic challenges.
- Surgical intervention remains the primary treatment modality for all ependymoma cases.
Implications:
- Close clinical evaluation and vigilant follow-up are essential for young children diagnosed with ependymomas, particularly those with grade II tumors.
- This case underscores the importance of monitoring for anaplastic transformation in pediatric ependymomas.
- Individualized treatment strategies and intensive follow-up protocols are necessary for improving outcomes in young infants with CNS neoplasms.
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