Long-Term Outcomes After Multidisciplinary Treatment for Pediatric Orbital Rhabdomyosarcoma.

(1)

Background

Orbital rhabdomyosarcoma is a rare and aggressive soft tissue tumor that primarily occurs in the eye socket (orbit) of children. Treatment usually involves a combination of surgery, chemotherapy, and radiation therapy, aiming to remove the tumor and prevent metastasis. (2)

Methods

An institutional retrospective study was conducted with data from 39 patients with primary orbital RMS treated between 1995 and 2016 at the Amsterdam University Medical Centers/Emma Children Hospital. (3)

Results

The median age at presentation was 7 years (range, 9 months to 16 years). The median follow-up period was 9.4 years (range, 3 to 25 years). Ten underwent chemotherapy and excision without additional radiotherapy. A total of 29 patients received additional local treatment: Ablative surgery MOld technique with after loading brachytherapy and surgical REconstruction (AMORE) (N = 21), proton (N = 4) or external beam radiation treatment (EBRT; N = 4). We found 14 cases with recurrences, 9 of which underwent exenteration and two of which died. The 10-year overall survival rate was 95% and the EFS was 63%. (4)

Conclusions

long-term follow-up with 10-year survival rate of orbital RMS in this series was 95% achieved by local tumor control and eye preservation in 77% of our study population.