| 발표일자: |
2013년 11월 1일(금) ~ 11월 3일(일) |
| 발표번호: |
P(판넬)-080 |
| 발표장소: |
킨텍스 제2전시장 7B홀 |
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| Primary intracranial myopericytoma presenting with multiple cranial nerve palsies. |
| Singapore National Eye Centre(1), Singapore Eye Research Institute (2), Singapore General Hospital (3) |
| Wong Chee Wai(1,2), Kesavan Sittapalam(3), Loo Jing Liang(1,2) |
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Purpose : To describe a rare case of primary intracranial myopericytoma presenting with multiple cranial nerve palsies.
Methods : Case report
Results : A 52 year old Chinese female presented with a 6 month history of left partial ptosis and downward deviation of the left eye. Clinical examination revealed left optic neuropathy, 10 prism diopters of left hypotropia and 16 prism diopters of left estotropia in primary position secondary to a pupil involving partial third cranial nerve palsy, fourth and sixth cranial nerve palsies. Magnetic resonance imaging of the brain with contrast showed a large avidly enhancing mass, 5cm in diameter, in the right middle cranial fossa, exerting mass effect on the right cavernous sinus and right intracranial optic nerve. She underwent left temporal craniotomy and excision of the mass and histology confirmed a diagnosis of primary intracranial myopericytoma. Post-operatively, the patient suffered infarcts of the left basal ganglia and middle cerebral artery, resulting in right hemiplegia and aphasia.
Conclusion : Primary intracranial myopericytoma (PIM) is an exceedingly rare benign tumor with only 3 previously described cases in the literature. We report the largest sized and most extensive case of PIM in the literature to date. All cases occurred in women aged 50-60 years old. Surgical management is fraught with danger owing to its tendency for perivascular growth. Further studies are needed to determine the natural history and optimal treatment for this condition.
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