Clear cell sarcoma of tendons and aponeuroses actually is a well identified soft tissue tumor. A primary orbital involvement is presented, with an optical and electron microscopic study. The orbital topography of such a tumor seems to be an exceptional opportunity. The histological study showed the typical pattern of this clear cell tumor. Ultrastructurally, there are not absolute specific features to establish the diagnosis, except the finding of intra-cellular melanosomes. Histogenesis has been debated for a long time between a melanotic or a synovial origin. According to present knowledge, this tumor may be attributed to a neural crest origin.
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