Tylectomy Safety in Salvage of Eyes with Retinoblastoma

Abstract

Intraocular surgery is tabooed in retinoblastoma management, due to the concern of lethal extraocular spread. We reviewed the outcomes of consecutive children with intraocular retinoblastoma diagnosed at 29 Chinese centers between 2012-2014. We compared the outcomes of three categories of treatment: eye salvage including tylectomy (Group I), eye salvage without tylectomy (Group II), and primary enucleation (Group III). A total of 960 patients (1243 eyes) were diagnosed: 256 in Group I, 370 in Group II, and 293 in Group III; 41 patients abandoned treatment upfront. The estimated 5-year overall survivals (OS) were, for Group I, 94%, for Group II 89%, and for Group III 95%. The estimated 5-year disease-specific survivals (DSS) were, for Group I, 96%, for Group II 90%, and for Group III 95%. Patients in Group I had a significantly higher 5-year DSS than patients in Group II (p = 0.003) and not significantly different than patients in Group III (p = 0.367). Overall survival was not compromised by the inclusion of tylectomy in eye salvage therapy compared to eye salvage without tylectomy or primary enucleation. Disease-specific survival was better when tylectomy was included in eye salvage treatments. Tylectomy as part of multimodal treatment may contribute to the care of retinoblastoma patients with chemotherapy-resistant tumor, eyes with concomitant ocular complications, or at the risk of treatment abandonment.

Keywords: endoresection; enucleation; pars plana vitrectomy; resection; retinoblastoma; safety; surgery; survival; tylectomy.

Figure 1

Standard care for retinoblastoma by IIRC Group leading to eligibility for tylectomy. IVC, intravenous chemotherapy; IAC, intra-arterial chemotherapy.

Figure 2

(A) Images and treatment timeline of patient #505 treated with tylectomy for refractory tumor who achieved good visual outcome. The patient was diagnosed with unilateral Group D retinoblastoma in the right eye at 2 years of age. Following 6 cycles of systemic chemotherapy, there was still a significant active tumor inferior to the optic disc and vitreous seedings. A tylectomy was performed to remove all active tumors inside the eye. One cycle of adjuvant systemic chemotherapy was given 2 days following tylectomy. There was no tumor recurrence at 70-month follow-up with visual acuity tested 20/100. IVC, intravenous chemotherapy; Ivitc, intravitreal chemotherapy. (B) Images and treatment timeline of patient #607 treated with tylectomy for refractory tumor who developed anterior chamber tumor recurrence. The patient was diagnosed with unilateral Group D retinoblastoma in the left eye at 2 years of age. Following 3 cycles of systemic chemotherapy, there was a persistent large active tumor in the temporal retina, diffuse subretinal seedings, and serous retinal detachment. The parents declined enucleation. A tylectomy was performed to remove all active tumors inside the eye. Five months following tylectomy, the eye developed anterior chamber tumor recurrence. The eye was enucleated and had pT3a histopathology. The child received 4 cycles of systemic chemotherapy. There was no evidence of orbital recurrence at 67 months follow-up. IVC, intravenous chemotherapy; Enu, enucleation; AC, anterior chamber.

Figure 3

Kaplan–Meier curves of disease-specific survival (DSS) of patients from Group I, II, and III.