A 20-year audit of retinoblastoma treatment outcomes

Abstract

Objectives To evaluate the long-term treatment outcomes in intraocular retinoblastoma (RB) including the associated factors for eventual treatment with external beam radiotherapy (EBRT) and enucleation as well as to analyse the risk factors for metastasis and death in extraocular RB. Methods Retrospective analysis of 390 eyes from 256 (89.8%) intraocular RB and 29 (10.2%) extraocular RB cases diagnosed and treated between October 1998 and May 2018 at one of the largest tertiary care centers in Turkey. Results Of 351 intraocular RB eyes, 53.3% had group D/E disease at presentation. 75 (21.4%) of 351 eyes underwent primary enucleation. Of the remaining 276 eyes undergoing eye-conserving treatments, 201 (72.8%) were salvaged. Most of these eyes were treated using intravenous chemotherapy and/or focal treatments [transpupillary thermotherapy (TTT) and cryotherapy] initially. EBRT was eventually required in 48 (17.4%) eyes and secondary enucleation in 75 (27.2%) eyes. At mean follow-ups of 76.7 and 39.7 months for intraocular and extraocular RB cohorts, respectively, 180 (46.2%) eyes underwent primary/secondary enucleation and exenteration. Overall, 13 cases developed metastasis and 9 died. Two patients with trilateral RB also expired. Multivariable risk factors for enucleation were the presence of vitreous seeds ( p < 0.001), absence of EBRT administration ( p = 0.033), 5–9 TTT applications compared with no TTT ( p = 0.031), and each 1 mm increase in tumour base diameter ( p < 0.001). Univariate factors predictive of metastasis were the presence of extraocular RB detected by imaging methods ( p < 0.001) and extrascleral/optic nerve cut end involvement at histopathological examination ( p < 0.001). Conclusions In our series, 72.8% of the intraocular RB eyes undergoing eye-conserving treatments were saved. The globe salvage rate for all intraocular and extraocular RB eyes was 53.8% and the overall survival rate was 96.1%.

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

“…Although more studies with longer follow-ups are needed to be able to reach a conclusion, such fears have not, however, been confirmed to date. Indeed, when considering the studies reporting on metastatic disease, a similar metastasis rate of about 2% is found in children treated with systemic chemotherapy [ 15 , 16 , 19 , 22 , 25 , 26 ] or intra-arterial chemotherapy [ 23 , 29 , 33 , 34 , 35 , 37 , 38 , 42 ], whereas, according to a retrospective multicentric survey including more than 1100 patients managed with primary ( n = 464) or salvage intra-arterial chemotherapy ( n = 713) over a 10-year period, the risk of metastatic death from retinoblastoma has been estimated to be less than 1% [ 89 ].…”

“…Since the beginning of conservative retinoblastoma management, the main reason to stop eye-preserving therapies has always been, and still is related, independently of the treatment modalities used, to uncontrolled tumor activity (80–90% of SE) [ 2 , 4 , 5 , 6 , 7 , 10 , 11 , 13 , 14 , 15 , 16 , 17 , 18 , 19 , 20 , 21 , 22 , 23 , 24 , 26 , 27 , 29 , 30 , 31 , 32 , 34 , 35 , 37 , 38 , 40 , 41 , 53 , 54 , 55 , 56 ], while the occurrence of intraocular complications, especially those obscuring the fundus view, explains the remaining cases (10–20% of SE) [ 2 , 5 , 10 , 11 , 13 , 16 , 17 , 18 , 21 , 23 , 28 , 29 , 34 , 39 , 40 , 41 …”

“…Despite the significant advances made in retinoblastoma management over the last decades, secondary enucleation (SE) is sometimes inevitable in order to preserve the patient from metastatic disease and death, and remains to date the treatment of choice for eyes that did not respond favorably to conservative strategies. Current indications to discontinue eye-preserving therapies include progressive/relapsing disease [ 1 , 2 , 3 , 4 , 5 , 6 , 7 , 8 , 9 , 10 , 11 , 12 , 13 , 14 , 15 , 16 , 17 , 18 , 19 , 20 , 21 , 22 , 23 , 24 , 25 , 26 , 27 , 28 , 29 , 30 , 31 , 32 , 33 , 34 , 35 , 36 , 37 , 38 , 39 , 40 , 41 , 42 ], persistent disease obscuring the optic nerve head, loss of fundus view (secondary to poor pupillary dilatation, intraocular hemorrhage and/or cataract) [ 2 , 5 , 17 , 18 , 40 , 41 , 43 , 44 ], neovascular complications [ …”

Global retinoblastoma survival and globe preservation: a systematic review and meta-analysis of associations with socioeconomic and health-care factors

“…The pooled estimate in 2010-20 was 6% (0-20, I²=98%) for LICs, 34% (21-49, I²=99%) for LMICs, 47% (38-56, I²=96%) for UMICs, and 70% (60-80, I²=96%) for HICs (table 2; appendix p 8). Globally, the pooled estimate improved significantly, from 22% [14][15][16][17][18][19][20][21][22][23][24][25][26][27][28][29][30][31][32] in 1980-89 to 44% [36][37][38][39][40][41][42][43][44][45][46][47][48][49][50][51][52] in 2010-20 (p=0•017).…”

Plumbagin Induces Cytotoxicity via Loss of Mitochondrial Membrane Potential and Caspase Activation in Metastatic Retinoblastoma

“…The long-term survival rates of patients with metastatic RB remain unsatisfactory, mainly a result of invasion and metastasis (5). The frequency of metastatic RB ranges from 4.8 to 11% (6,7) and it remains major contributor to mortality and is associated with life-long poor prognosis (6,(8)(9)(10)(11). Frequent common sites of extraocular RB include the orbit, pre-auricular nodes, bones, central nervous system and liver, and each of these may influence the outcome of metastatic RB.…”