This protocol addresses bilateral retinoblastoma in patients with a confirmed germline RB1 mutation or a family history of retinoblastoma — a population with hereditary predisposition requiring specific management considerations. The cases in scope do not exhibit high-risk features such as extraocular extension or significant choroidal or optic nerve invasion.
Prior globe-sparing interventions — which may include intra-arterial chemotherapy (IAC), intravitreal chemotherapy, consolidation cryotherapy, thermotherapy (TTT), plaque radiotherapy, or intracameral chemotherapy — are pursued first to preserve the eye. This protocol is reached when those measures have failed to achieve control and resolution of vitreous and subretinal seeds with tumor regression.
DOI: 10.4103/ijo.IJO_721_20
Current indications for IVC include patients with bilateral disease, confirmed germline mutation, family history of retinoblastoma, or cases with suspected optic nerve or choroidal invasion.
It is usually reserved for massive group E tumors, poor tumor visualization (e.g., due to vitreous hemorrhage), presence of extraocular extension, suspected invasion of the optic nerve or choroid, or recalcitrant tumors that have failed previous globe salvage therapies (e.g., IAC, IvitC, plaque radiotherapy, etc.).
High risk retinoblastoma warrants enucleation and additional 6‑9 cycles of high‑dose IVC to prevent metastatic disease.