안구아탈구를 유발한 거대 소아 털모양별아교세포종 1예
A Case of Huge Pilocytic Astrocytoma Causing Eyeball Subluxation
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목적: 8세 남아에서 시신경과 시신경 교차부의 거대 털모양별아교세포종(pilocytic astrocytoma)으로 인하여 안구아탈구가 발생한 1예를 경험하였기에 보고하고자 한다. 증례요약: 8세 남아가 1년 전부터 급격히 진행한 좌측 안구돌출 및 시력 소실을 주소로 내원하였다. 영상학적 검사에서 2.9×2.7×4.2cm 크기의 종괴가 좌측 시신경 및 시신경 교차부에 위치하고 있음이 발견되었다. 진단 및 치료를 위해 종양절제술과 안구적출술을시행하였다. 병리 조직 검사에서 성상세포종(astrocytic Tumor) 중 세계보건기구(World Health Organization, WHO)의 분류 1단계에해당하는 털모양별아교세포종(pilocytic astrocytoma)으로 확인되었다. 결론: 성상세포종은 소아 연령에서 가장 흔한 뇌종양이며 이 중에서 털모양별아교세포종(pilocytic astrocytoma)은 비교적 비침윤성경향을 보이기 때문에 종양제거술만으로 완치가 기대 가능한 종양이다. 소아에서 급격히 진행하는 안구돌출 및 시력저하 시 발생할수 있는 안와종양으로 성상세포종을 감별진단에 고려하여야 할 것이다.
Abstract
Purpose: To report a relatively rare case of huge pilocytic astrocytoma of the optic nerve and optic chiasm causing eyeballsubluxation. Case summary: An eight-year-old male presented with proptosis and visual loss in the left eye for one year. The radiological findingsshowed a 2.9 x 2.7 x 4.2-cm tumor on the left optic nerve and optic chiasm. For diagnosis and treatment, the patient underwenttumor resection and enucleation. Pathohistological analysis of the tumor specimen revealed pilocytic astrocytoma, which isclassified by the World Health Organization as a grade I astrocytic tumor. Conclusions: Astrocytoma is a tumor of the brain that affects children more often than adults. In general, gross-total resection ofpilocytic astrocytoma is expected to be curative due to the non-invasive feature of the tumor. Considering pilocytic astrocytomaas differential diagnosis of orbital tumor in children with symptoms of rapidly progressive proptosis and decreased visual acuityis important because occurrence in the optic nerve and optic chiasm is possible.
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