An Unexpected Response of Orbital Retinoblastoma to Inconsistent Systemic Chemotherapy Alone.

Introduction: A 16-month-old girl presented with bilateral germline retinoblastoma, with advanced retinoblastoma in the right eye and severe orbital retinoblastoma in the left eye. Patients typically receive treatment with the multimodal protocol consisting of chemotherapy, surgery, and radiotherapy, which has improved survival.

Case Presentation: Our patient elected for medical management with systemic chemotherapy instead of enucleation or exenteration. She received three blocks systemic chemotherapy consisting of vincristine sulfate, etoposide phosphate, and carboplatin, with irregular intervals between blocks over 3 years, due to poor compliance. Following chemotherapy, she had tumor regression in both eyes and no signs of active disease 13 years later.

Conclusion: This case represents an interesting favorable outcome of orbital retinoblastoma management with systemic chemotherapy alone despite inconsistency versus conventional multimodal protocol.