Background: Although rhabdomyosarcoma (RMS) is a rare tumor among the entire group of mesenchymal malignancies, it is a relatively common lesion and significant challenge for the ocular oncologist in terms of its diagnosis and management. Methods: A comprehensive literature search of articles published over the past 30 years in PubMed was conducted. Results: Orbital RMS usually presents as a space-occupying lesion in the orbit during the first decade and may mimic other neoplastic or inflammatory masses. The tumor has predilection for the superior nasal quadrant of the orbit. The clinical manifestations depend on the location of the tumor within the orbit and its rate of growth. The common histopathologic types are embryonal and alveolar varieties. CT and MR imaging are important in the evaluation of this tumor. Particular attention should be placed on the bone invasion and extension of the tumor into the intracranial cavity and paranasal sinuses. Treatment usually consists of a combination of chemotherapy and radiation therapy following excisional biopsy. Conclusions: Survival of orbital RMS has improved due to advances in chemotherapy and radiotherapy. Posttreatment complications, including side effects of radiotherapy and secondary orbital malignancies, as well as visual dysfunction, occur more often and present new challenges due to improved long-term survival.
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