Rhabdomyosarcoma: review for the ophthalmologist

Rhabdomyosarcoma is a malignant neoplasm that is composed of cells with histopathologic features of striated muscle in various stages of embryogenesis. It can occur in several sites in the body, including the ocular region. Ocular rhabdomyosarcoma is defined as the occurrence of this tumor in the area of the eye. Most ocular rhabdomyosarcomas arise in the soft tissues of the orbit but they can rarely occur in the other ocular adnexal structures and even within the eye. The purpose of this review is to provide a brief overview of rhabdomyosarcoma and a more detailed review of orbital rhabdomyosarcoma, with emphasis on changing concepts in the diagnosis and management of this ophthalmic neoplasm.