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ANAPLASTIC EPENDYMOMA RECURRENCE IN PEDIATRIC POPULATION: WHAT TO DO?
Case Presentation, Discussion, and Final Comments.
Patient, male, 7 years old, previously higid, presenting behavior change, nausea and vomits, accessory palsy, diplopy, dismetry, for two weeks, has been submitted to Magnetic Resonance that showed lesion in the IV ventricle expanding to Luschka Forame and compressing the brainstem in pre bulbar cistern. He Has been operated by Telovelar via with total ressection, without new deficits. Microscopy showed glial neoplasm consisting of ependymal cells, microvascular proliferation, and some foci of necrosis. ki67: 25%. The histopathologic diagnosis was Anaplastic Ependymoma WHO GRADE III. Submitted to 54 gy dose Radiotherapy in 30 session. Within 2 years, there was more 2 surgery to recurrent lesion by telovelar and retrosigmoid via . Presenting residual lesion averege 1.5 cm3 in floor IV ventricle. Clinically in good general state, facial paresis House- Brackmann grade II, studying, playing sports, without headache, without diplopy.
Ependymoma is the third most common CNS tumor in children . For localized ependymomas, the standard treatment is surgery followed by focal irradiation of the primary tumor site. However, despite aggressive treatment, one-third of patients experience recurrence. Tumors that expand to Luschka Forame and close to the brainstem should be a challenge and, in most cases, total resection is dificult. Retrospective studies have shown re-treatment of ependymoma with a second course of fractionated radiation therapy to be effective. Furthermore, the role of molecular markers associated with prognosis in de novo ependymoma, such as gain of chromosome 1q is unknown in patients selected for re-treatment. The optimal treatment volume for re-irradiation is unclear. Focal re-irradiation may be used at the time of recurrence for local recurrence, but it is associated with important late toxicities, especially neurocognitive impairment.
Then residual lesion, even though surgery and local radiation, create a question : re-radiate a tumor site? Re-operate ?
Anaplastic Ependymoma recurrence is common. In this case, there was residual lesion close to the brainstem, in the floor of IV ventricle, after three surgeries and a radiotherapy session. Due to difficulty in surgical re-approach and radiotherapy risks, the question arises of “ how to handle the case?”
GABRIEL MUFARREJ NETO, PAULO DE TARSO FONSECA MARQUES CASTRO