Modern treatment of retinoblastoma: A 2020 review

Abstract

Retinoblastoma management remains complex, requiring individualized treatment based on International Classification of Retinoblastoma (ICRB) staging, germline mutation status, family psychosocial factors and cultural beliefs, and available institutional resources. For this 2020 retinoblastoma review, PubMed was searched for articles dated as early as 1931, with an emphasis on articles from 1990 to the present day, using keywords of retinoblastoma, chemotherapy, intravenous chemotherapy, chemoreduction, intra-arterial chemotherapy, ophthalmic artery chemosurgery, intravitreal chemotherapy, intracameral chemotherapy, cryotherapy, transpupillary thermotherapy, laser, radiation, external beam radiotherapy, plaque radiotherapy, brachytherapy, and enucleation. We discuss current treatment modalities as used in the year 2020, including intravenous chemotherapy (IVC), intra-arterial chemotherapy (IAC), intravitreal chemotherapy (IvitC), intracameral chemotherapy (IcamC), consolidation therapies (cryotherapy and transpupillary thermotherapy [TTT]), radiation-based therapies (external beam radiotherapy [EBRT] and plaque radiotherapy), and enucleation. Additionally, we present a consensus treatment algorithm based on the agreement of three North American retinoblastoma treatment centers, and encourage further collaboration amongst the world's most expert retinoblastoma treatment centers in order to develop consensus management plans and continue advancement in the identification and treatment of this childhood cancer.

Keywords: Algorithm; eye; oncology; pediatric; retinoblastoma; treatment.

Figure 1

Modern treatment of retinoblastoma. The role of intravenous chemotherapy (IVC) in bilateral disease. A 4-month-old patient was diagnosed with a (a) Group B retinoblastoma in the right eye, and was treated with 6 cycles of standard-dose IVC, (b) achieving a complete regression of the tumor. Consolidation therapy with TTT was required during the course of the treatment, leaving flat scars (black arrows) and completely regressed tumors. The (c) left eye was diagnosed with Group D retinoblastoma, regressing to a (d) smaller calcified scar in the macular region after treatment

Figure 2

Modern treatment of retinoblastoma. The role of intra-arterial chemotherapy (IAC) in unilateral disease. (a) Unilateral group B retinoblastoma with macular involvement. Following 4 cycles of IAC, (b) the majority of the macula had been spared without the need for additional consolidation therapies. (c) Unilateral group D retinoblastoma with macular involvement and serous retinal detachment. After 4 cycles of IAC, the retina completely reattached leaving a (d) smaller calcified macular scar and scattered calcified subretinal seeds

Figure 3

Modern treatment of retinoblastoma. The role of intraocular chemotherapy. Diffuse vitreous seeding from retinoblastoma managed with intravitreal chemotherapy (IvitC). (a) Active vitreous seeds surrounding the tumor and overlying the macula (black arrow), with (b) resolution after two cycles of IvitC with melphalan and one cycle of IvitC with topotecan

Figure 4

Modern treatment of retinoblastoma. The role of consolidation therapies. Group A retinoblastoma managed with transpupillary thermotherapy (TTT). (a) Subtle tumor (black arrow) temporal to the macula, with (b) regression 1 month after treatment

Figure 5

Modern treatment of retinoblastoma. The role of enucleation and prosthetic rehabilitation. (a) Unilateral Group E retinoblastoma that required (b) enucleation, with a dermo-lipid graft placed for economic reasons. (c) On follow-up 6 weeks later, a custom-made prosthesis was adjusted