Retinoblastoma
ICD-10 C69.2 · ICD-11 2D02.2

Non-Germline Unilateral Retinoblastoma (ICRB Group B–E): Next-Line Protocol After Globe Salvage Therapy Fails to Control Seeds

This protocol applies to non-germline unilateral retinoblastoma classified as ICRB Group B with macular involvement, or Group C, D, or E — in the specific setting where prior globe salvage therapy has not achieved control and resolution of vitreous and subretinal seeds, and where high-risk features such as extraocular extension or significant choroidal or optic nerve invasion are absent.

Clinical Scenario

Non-germline unilateral retinoblastoma, ICRB Group B (macular involvement) through Group E, without high-risk histopathological features. The defining characteristic of this patient group is that globe salvage has been pursued but seed control has not been reached, making escalation necessary.

Previous Line — Why This Protocol Is Reached

Escalated globe salvage therapy has been attempted — including approaches such as consolidation cryotherapy, transpupillary thermotherapy (TTT), intra-arterial chemotherapy (IAC), plaque radiotherapy, intravitreal chemotherapy (IvitC), precision intravitreal chemotherapy (p-IvitC), and intracameral chemotherapy (IcamC) — without achieving the required goal: control and resolution of vitreous and subretinal seeds with tumor regression. This failure of seed control is the threshold that escalates to the current protocol.

Treatment Approach (Overview — Partial)

The central intervention is enucleation of the affected eye. Whether additional systemic chemotherapy is indicated depends on histopathological findings identified at surgery — the complete criteria and management algorithm are available in the full structured protocol.

Instant Access to Structured Evidence-Based Regimens

References

DOI: 10.4103/ijo.IJO_721_20

  • IAC is employed as primary therapy for non-germline, unilateral, group B, C, D, or E retinoblastoma or as a secondary therapy for unilateral or bilateral advanced recalcitrant disease facing enucleation.
  • Hence, the authors prefer IAC over IVC for unilateral retinoblastoma.
  • 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.
  • In the presence of high risk features, including post-laminar optic nerve invasion, massive choroidal invasion (>3 mm diameter), or extraocular extension, adjuvant IVC is required for prevention of metastases.
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