Current Indications of Secondary Enucleation in Retinoblastoma Management: A Position Paper on Behalf of the European Retinoblastoma Group (EURbG)

Abstract

Secondary enucleation (SE) puts an irreversible end to eye-preserving therapies, whenever their prolongation is expected to violate the presumed state of metastatic grace. At present, it must be acknowledged that clear criteria for SE are missing, leading to empiric and subjective indications commonly related to disease progression or relapse, disease persistence masking the optic nerve head or treatment-related complications obscuring the fundus view. This absence of evidence-based consensus regarding SE is explained by the continuously moving frontiers of the conservative management as a result of diagnostic and therapeutic advances, as well as by the lack of studies sufficiently powered to accurately stratify the risk of metastasis in conservatively treated patients. In this position paper of the European Retinoblastoma Group (EURbG), we give an overview of the progressive shift in the indications for SE over the past decades and propose guidelines to assist decision-making with respect to when SE becomes imperative or recommended, with corresponding absolute and relative SE indications. Further studies and validation of biologic markers correlated with the risk of metastasis are expected to set more precisely the frontiers of conservative management and thus consensual criteria for SE in the future.

Retinoblastoma treatment in a Brazilian population. Presentation and long‐term results

“…26 It is possible that in these patients, surgery could be avoided. 23 Otherwise, postlaminar optic nerve invasion (stage pT3b) was related to the presence of goniosynechiae, supporting the indication of enucleation in these cases. In fact, it seems that many retinoblastomas cause ischemia and neovascularization before invading the optic nerve or choroid.…”

Advances in biomaterials for the treatment of retinoblastoma

“…In spite of being rarely performed in early retinoblastoma, various studies describe success rates of saving the eye from advanced retinoblastoma ranging from 29% to 74% when treated with systemic chemotherapy and 0% to 61% when treated with intra-arterial chemotherapy. 92,93 The most commonly used chemotherapeutic drugs for the treatment of retinoblastoma include carboplatin, vincristine, etoposide, cyclophosphamide, topotecan, and doxorubicin, among others. 94,95 Surgical enucleation: The removal of the eye is the last treatment option that is used by surgeons to prevent metastasis and for the preservation of life.…”

Automatic Retinoblastoma Screening and Surveillance Using Deep Learning

“…3 For globe salvage, almost all retinoblastoma classified by the International Intraocular Retinoblastoma Classification (IIRC) 4 as group A-C can achieve globe salvage through systemic chemotherapy with adjuvant laser therapy and cryotherapy. 5 Group D retinoblastoma can also achieve 40% (95%CI:31–51%) globe salvage rate through chemotherapy alone 6 and a significantly higher globe salvage rate when receiving intra-ophthalmic artery chemotherapy. 7…”