眼窩先端部症候群と顔面神経麻痺を生じた悪性リンパ腫の1例

A case of malignant lymphoma presenting as orbital apex syndrome and facial nerve palsy

要約　目的：眼窩初発で経過中に中枢神経系以外の臓器（頸部リンパ節）に生じた，びまん性大細胞型B細胞性リンパ腫（DLBCL）の症例報告。

症例：88歳，女性。左側動眼神経不全麻痺による複視と眼瞼下垂が出現するも自然軽快した。数か月後に，左側の外転神経，動眼神経，滑車神経，三叉神経，視神経，顔面神経障害による症状が出現した。造影T1強調MRI画像で左眼窩，海綿静脈洞，中頭蓋窩に病変が検出された。右鎖骨上窩リンパ節生検による病理組織検査で，DLBCL（アン・アーバー病期分類：Stage ⅡA）と診断され，化学療法が施行されたが，間質性肺炎を併発し死亡した。

結論：多発脳神経障害をきたす眼窩および海綿静脈洞病変では，悪性リンパ腫も念頭に置き，積極的に全身病変の検索を行うべきである。

Abstract　Objective：To report a case of diffuse large B-cell lymphoma（DLBCL）that began in the orbit and resulted in multiple cranial nerve impairment：the cervical lymph node（except for the central nervous system）was involved in the course of disease.

Case：The patient was an 88-year-old woman who developed ptosis and diplopia caused by left oculomotor nerve palsy and then recovered. After five months, she showed vertical diplopia, seemingly sagging eye syndrome, which progressed to multiple cranial nerve impairment involving the Ⅵ, Ⅲ, Ⅴ1-3, Ⅳ, Ⅶ, and Ⅱ cranial nerves of the left side. Post-contract T1-weighted MRI revealed enhancement of the left orbit, cavernous sinus, and middle cranial fossa. Histopathological examination of the right supraclavicular lymph node biopsy was performed, and the patient was diagnosed with DLBCL（Ann Arbor：Stage ⅡA）.

Conclusion：In the presence of multiple cranial nerve impairment involving the orbit and cavernous sinus, malignant lymphoma should be considered and aggressively examined for systemic involvement.