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KMID : 0942820070060020120
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Journal of Korean Brain Tumor Society
2007 Volume.6 No. 2 p.120 ~ p.124
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Atypical Teratoid/Rhabdoid Tumor in Infant - Case Report -
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Moon Jong-Un
Kim Tae-Sung
Park Bong-Jin
Lim Young-Jin
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Abstract
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Atypical teratoid/rhabdoid tumor(AT/RT) is extremely rare and highly malignant tumor of early childhood. AT/RT shows similar histopathological and radiological features to primitive neuroectodermal tumor(PNET) but more aggressive behavior than PNET. We report a case of supratentorial AT/RT with review of literatures. A 11-month-old female presented with a 2-weeks history of left side hemiparesis and convulsion. On neurologic examinations, there were no specific findings on developments and activities, except left side hemiparesis and intermittent seizure. Brain magnetic resonance images(MRI) revealed PNET such as primary cerebral neuroblastoma at right frontotemporal lobe with midline shift to the left side and suggestive of seeding nodule at occipital horn of left lateral ventricle. There were no evidences of spinal metastasis. Right frontotemporal mass was subtotally removed. Symptoms and signs were mildly improved but seizure was ontinued. Histopathological examination showed findings of AT/RT. In spite of our recommendation of radiation therapy and chemotherapy, her parents refused further treatment. On postoperative brain computed tomography(CT) scan, subtotal removal state of right frontotemporal lobe mass with marked reduction of mass effect and midline shift were shown. On follow-up radiologic examinations at postoperative 4 months, residual tumor was grown and hydrocephalus was progressed aggressively. AT/RT of the cerebrum is an extremely rare and aggressive tumor of early childhood. We suggest that aggressive management may be required even though tumor should be progressed rapidly and shows poor prognosis.
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KEYWORD
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Atypical teratoid/Rhabdoid tumor, Primitive neuroectodermal tumor, Medulloblastoma
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