Topic outline

  • Grand Rounds

    A young patient with notable past ocular history of orbital rhabdomyosarcoma, s/p chemotherapy with vincristine, dactinomycin, and cyclophosphamide, and proton beam radiation presented to Bascom Palmer ED with 2 days of blurry vision and “seeing everything green” OD. The patient was imaged and found to have a soft tissue lesion in the posterior inferior orbit OD extending through superior orbital fissure and mild displacement of the optic nerve. On biopsy, the patient was found to have recurrence of rhabdomyosarcoma. Orbital rhabdomyosarcoma is the most common malignant orbital mass in pediatrics and usually presents with rapid onset and severe proptosis/globe displacement. Though the survival rate of ORMS is excellent, it can recur in up to 17% of cases. Recurrence is treated in a similar way to initial malignancy with a combination of resection, chemotherapy, and radiation therapy, however there are no clear guidelines on the management of recurrence, and it usually based on the clinical judgement of the physician managing the case.

    Presentation Date: 08/24/2023
    Issue Date: 09/01/2023