Management of massive orbital involvement of intraocular retinoblastoma

Objective: This study aimed to evaluate various combinations of therapeutic approaches in an attempt to improve the poor outcome of advanced cases of orbital involvement of intraocular retinoblastoma.

Design: The study design was a retrospective, uncontrolled descriptive case series of consecutive patients with massive orbital retinoblastoma treated and observed at a referral center.

Participants: Sixteen patients with unilateral orbital involvement of intraocular retinoblastoma were studied. Eight patients had orbital involvement at initial presentation and eight children presented with orbital tumor after enucleation for intraocular retinoblastoma. Patients having microscopic evidence of tumor invasion at surgical section of the optic nerve and subsequently receiving other forms of treatment were not included in the study.

Intervention: Six patients had orbital exenteration followed by external beam radiation therapy (4600-6000 cGy in fractionated doses) and chemotherapy. Five patients received chemotherapy only. Three patients were treated by exenteration and chemotherapy, whereas two patients underwent radiation therapy and chemotherapy.

Main outcome measures: Tumor regression monitored by clinical observations and imaging studies and patient survival was studied.

Results: The median follow-up was 22 months (range, 4-168 months). All forms of treatment caused satisfactory local and systemic tumor regression. Two children achieved a survival of more than 10 years. However, two patients in the exenteration followed by chemotherapy and radiation therapy group and one who received chemotherapy only had central nervous system metastases develop and died. Only one patient was lost to follow-up.

Conclusion: Highly individualized aggressive treatment by combining radical surgery, chemotherapy, and external beam radiation therapy allowed most of the authors' patients to have longer survival and disease-free intervals than expected.