Tylectomy Safety in Salvage of Eyes with Retinoblastoma

SIMPLE SUMMARY: The role of organ-conserving surgery has not been explored in retinoblastoma as it has been in other cancers, such as breast cancer lumpectomy, partial nephrectomy for kidney cancer, and partial orchiectomy for testis cancer. This is largely accounted for by the high mortality of extraocular retinoblastoma compared to intraocular retinoblastoma, and fear of iatrogenic tumor spread with intraocular surgery. We propose the little-known word “tylectomy” (“tulos”, Greek for “lump”) to describe the surgical resection of retinoblastoma. Through review of consecutive patients treated by our team between 2012–2014, we compared survival of patients with eye salvage, including tylectomy, to those who had eye salvage without tylectomy or primary enucleation. We found that patients who had tylectomy had superior survival compared to those who had eye salvage without tylectomy (96% vs. 90%), and comparable survival to those with primary enucleation (96% vs. 95%). Our study supports tylectomy as a safe contribution to retinoblastoma management. 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.