Treatment of Progressive or Recurrent Intraocular Retinoblastoma

Treatment options for progressive or recurrent intraocular retinoblastoma include the following:

For more information about the use and potential applications of intra-arterial and intravitreal chemotherapy, see the sections on Intra-Arterial Chemotherapy (Ophthalmic Artery Infusion of Chemotherapy) and Intravitreal Chemotherapy.

New intraocular tumors can arise in patients with the heritable form of disease whose eyes have been treated with local control measures only because every cell in the retina carries the RB1 variant. This event should not be considered a recurrence. Even with previous treatment consisting of chemoreduction and local control measures in very young patients with heritable retinoblastoma, surveillance may detect new tumors at an early stage. Additional local control therapy, including plaque radiation therapy, can successfully eradicate these tumors.[2]

When the recurrence or progression of retinoblastoma is confined to the eye and is small, the prognosis for sight and survival may be excellent with local therapy only.[3][Level of evidence C3] If the recurrence or progression is confined to the eye but is extensive, the prognosis for sight is poor; however, survival remains excellent.

Intra-arterial chemotherapy (IAC) into the ophthalmic artery has been effective in patients who relapse after systemic chemotherapy and radiation therapy.[4,5] Rescue IAC, usually with other agents, has also been used after primary IAC.[6] Plaque radiation therapy is an option for patients who have a retinoblastoma recurrence after IAC treatment.[7][Level of evidence C3] Radiation therapy should be considered for patients who have not been previously irradiated. Finally, enucleation may be required in cases of progressive disease after all eye-salvaging treatments have failed.