Abstract
Purpose :
Primary Ewing’s sarcoma of the orbit is quite rare. The purpose of this case series is to describe the clinical and radiologic features as well as treatments in 8 patients with primary Ewing’s sarcoma of the orbit.
Methods :
Patient records and radiologic files were retrospectively reviewed. Data collected included age, gender, ethnicity, AJCC 8th edition stage at presentation, size of tumor, radiologic features, treatments, and survival outcomes.
Results :
There were seven males and one female. The cohort consisted of 4 pediatric patients (less than 18 years of age) and 4 adult patients, with a median age of 14 years (range: 2 to 42 years). The most common initial presenting sign was proptosis. Six patients had tumors affecting the right eye. Treatment plan for six patients consisted of biopsy to confirm the diagnosis, and chemotherapy followed by, or with the addition of, radiation therapy. 4 patients were treated with intensity-modulated radiation therapy (IMRT), 3 patients were treated with proton therapy, and 1 was treated with both IMRT and proton therapy. All patients except for one received chemotherapy before radiation. Surgery was used as salvage (to remove tumor recurrence). None of the patients had orbital exenteration. Follow-up period ranged from 4 to 147 months (median = 37 months). At last follow up, seven patients had no evidence of disease, while one patient had died of disease.
Conclusions :
Primary Ewing’s sarcoma of orbit is sensitive to chemotherapy and radiation. With multi-modality treatment, eye salvage is possible in most patients with Ewing’s sarcoma of the orbit.
This is a 2020 ARVO Annual Meeting abstract.