Poorly differentiated primary orbital sarcoma (presumed malignant rhabdoid tumor). Radiologic and histopathologic correlation.

A 47-year-old man presented with acquired hyperopia and gaze-evoked visual loss. Computed tomography and magnetic resonance imaging demonstrated a well-circumscribed intraconal tumor. Histopathologic studies indicated that the tumor was a poorly differentiated primary orbital sarcoma compatible with malignant fibrous histiocytoma or malignant rhabdoid tumor. To our knowledge, no case of primary orbital malignant rhabdoid tumor has been previously described in an adult.