A narrative review of retinoblastoma and recent advances in its management

Abstract

Retinoblastoma (RB) is the most common malignant ocular tumor in children. The management of RB in the recent decades has become more advanced and personalised and has led to an improvement in the outcomes. The objective of this narrative review is to discuss the general overview, broad principles of management and recent advances in RB. Leucocoria is the most common manifestation of RB. Other manifestations are strabismus, painful blind eye, and loss of vision. RB is diagnosed late in the less-developed nations and it can present with spread to other adjacent structures like the optic nerve, orbit, brain, and systemic metastasis. The diagnosis of RB requires an examination of the eye under anesthesia (EUA). Imagings of the brain and orbits are also done to ascertain the spread. In cases of extraocular RB (EORB), a bone marrow examination and cerebrospinal fluid (CSF) studies are done to rule out the systemic spread. Life salvage, vision salvage followed by the globe's salvage should be the objectives in order of importance. Early lesions respond to focal therapy measures like cryotherapy, transpupillary thermotherapy (TTT), and laser photocoagulation. More advanced lesions need chemotherapy followed by focal therapy measures. RB in which the vision is not salvageable is best treated by enucleation of the eye. If there are high-risk lesions present in the enucleated specimen further therapy with chemotherapeutic agents and/or radiotherapy is needed. EORB is best treated by neoadjuvant chemotherapy followed by enucleation and radiotherapy. Other methods used in RB management are intravitreal chemotherapy, intraarterial chemotherapy, plaque brachytherapy, etc. In the case of refractory/recurrent disease, alternative chemotherapeutic agents and local control measures can be employed. Trilateral RB and metastatic RB may respond to high dose chemotherapy and autologous transplant. After treatment, the eyes have to be followed up for recurrence with regular EUA's. Follow up for the late effects of chemotherapy, ocular rehabilitation and psychosocial support are of paramount importance. RB is a very curable tumor with contemporary management. The multidisciplinary management for RB is the need of the hour in the countries where the outcome is sub-optimal so that more children achieve cure.