Rhabdomyosarcoma presumed metastatic to the orbit

Abstract

Background: Orbital metastasis from rhabdomyosarcoma is a rare disorder with a poor prognosis for long-term survival. Only one other detailed account of this disorder has appeared in the ophthalmic literature.

Methods: The authors report the clinical features of four patients with presumed orbital metastasis from alveolar and embryonal rhabdomyosarcoma.

Results: The most common ophthalmic manifestations of orbital metastasis from rhabdomyosarcoma in these patients included proptosis, reduced visual acuity, orbital pain, and motility disorders. Computed tomography documented orbital masses in all of the patients. In those patients with a primary tumor of the maxillary sinus, there was no evidence of direct extension into the orbit. Despite combination chemotherapy and radiation, all four patients died of their illness within 6 months of orbital metastasis.

Conclusions: Although uncommon, rhabdomyosarcoma should be considered in the differential diagnosis of metastatic tumors to the orbit in children and adults. Despite the poor prognosis, prompt diagnosis and palliative radiotherapy may improve the quality of life for these patients with terminal disease.