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Primary embryonic rhabdomyosarcoma of the orbit in a 11 year o | Forensic Pathologist

Primary embryonic rhabdomyosarcoma of the orbit in a 11 year old child.

Rhabdomyosarcoma is a rare childhood cancer with an estimated 250-350 new cases per year. The head and neck region and in particular, the orbit, represent a major anatomic site for RMS. Orbital RMS is the most common primary orbital malignancy in children with approximately 35 new cases per year. 

Rhabdomyosarcomas are composed of cells with histologic features of striated muscle in various stages of embryogenesis that can occur in several sites in the body, including the ocular region. Most ocular rhabdomyosarcomas arise in the soft tissues of the orbit but they can rarely occur in other ocular adnexal structures and even within the eye such as in this case!

The patient had a closed funnel RD, no masses visualized on USG, CT of the orbit and skull at the time. He returned to the clinic about 3 months later with a prolapsed tumor in the eye.