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緒言
1952年Christophersonら1)。よ,組織学的にきわめて特徴的であるが組織由来不明の軟部肉腫を12例集め,alveolar soft part sarcomaと命名した。それ以後,alveolar soft part sarcomaの報告は1973年までに世界で125例,うち本邦で24例みられるが2),四肢に発生するものが大多数であり,眼窩原発の報告はわずか数例である。著者らはきわめて稀な眼窩原発のalveolar soft partsarcomaの1症例を経験したので報告する。
Occurrence of alveolar soft part sarcoma in the orbit is extremely rare. The present paper describes the 4 th case in literature and the first case in Japan.
The patient, a 15-month-old girl, showed mild proptosis of the right eye when first seen. Pro-ptosis gradually advanced up to 20 mm (Her-tel) during the following 50 days. A rubbery firm mass was palpated through the lower lid. Papilledema, engorgement of the conjunctival vessels and secondary glaucoma became evide-nt. Since alveolar soft part sarcoma was sus-pected on biopsy, orbital exenteration includ-ing the tumor was performed 10 weeks after the initial examination.
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