Choroidal Infiltration by Retinoblastoma: Predictive Clinical Features and Outcome

Kaliki, Swathi; Tahiliani, Prerana; Iram, Sadiya; Ali, Mohammed; Mishra, Dilip Kumar; Reddy, Vijay Anand P.

doi:10.3928/01913913-20160719-03

Cited by 6 publications

(9 citation statements)

References 25 publications

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“…Specifically, for the patients with PLONI in a prospective study 21 from France, none of 12 patients had an extraocular relapse after receiving 4 cycles of a less intensive combination of carboplatin, etoposide, cyclophosphamide, and vincristine. In a retrospective study 23 of patients with PLONI, 5 of 71 (7%) had an extraocular relapse after receiving a regimen similar to that in the COG ARET0332 protocol. In the present series, only 1 of 35 patients with PLONI had an extraocular relapse, but an additional child died of toxic effects.…”

Section: Discussionmentioning

confidence: 99%

Treatment of Nonmetastatic Unilateral Retinoblastoma in Children

et al. 2018

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IMPORTANCE Multi-institutional collaborative studies that include large patient populations for the management of retinoblastoma with histopathological risk factors could provide important information for patient management. OBJECTIVE To evaluate the implementation of a strategy for the management of nonmetastatic unilateral retinoblastoma in children based on standardized diagnostic and treatment criteria. DESIGN, SETTING, AND PARTICIPANTS This single-arm prospective study applied a strategy based on a single-center experience. The setting was a multicenter study in Latin America (Grupo de America Latina de Oncologia Pediatrica [GALOP]). Participants were children with nonmetastatic unilateral retinoblastoma (staged with the International Retinoblastoma Staging System). The study opened on July 1, 2008, and closed on December 31, 2014. Follow-up was updated until June 30, 2017. INTERVENTIONS Stage 0 patients (without enucleation) were given conservative therapy without a protocol. Stage I patients (with enucleation and no residual tumor) were divided into a high-risk group (retrolaminar invasion and/or scleral invasion) and a low-risk group (all remaining patients). High-risk children received adjuvant chemotherapy with 4 alternating cycles of regimen 1 (cyclophosphamide [65 mg/kg/d] [plus sodium-2-mercaptoethane sulfonate], idarubicin hydrochloride [10 mg/m 2 /d], and vincristine sulfate [0.05 mg/kg/d]) and 4 cycles of regimen 2 (carboplatin [500 mg/m 2 /d, days 1 and 2] and etoposide [100 mg/m 2 /d, days 1-3]). Low-risk children did not receive adjuvant therapy. Children with buphthalmia received neoadjuvant and adjuvant chemotherapy for a total of 8 cycles. MAIN OUTCOMES AND MEASURES Probability of event-free survival (extraocular relapse and death from any cause were considered events). RESULTS Among 187 children registered in the study, 175 were evaluable (92 [52.5%] female; median age, 22 months; age range, 3-100 months). Forty-two were stage 0 children, 84 were stage I low-risk children, and 42 were stage I high-risk children; there were 7 children in the buphthalmia group. With a median follow-up of 46 months, the 3-year probability of event-free survival was 0.97 (95% CI, 0.94-0.99), and the probability of overall survival was 0.98 (95% CI, 0.94-1.00). Stage 0 patients had no events, stage I low-risk patients had 1 event (orbital relapse treated with second-line therapy), stage I high-risk patients had 2 events (1 central nervous system relapse and 1 death from sepsis), and the buphthalmia group had 1 event (orbital relapse, followed by central nervous relapse and death). CONCLUSIONS AND RELEVANCE Adjuvant therapy may be effective for high-risk unilateral retinoblastoma but is toxic, and neoadjuvant chemotherapy for buphthalmus appears feasible.

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Section: Discussionmentioning

confidence: 99%

Treatment of Nonmetastatic Unilateral Retinoblastoma in Children

et al. 2018

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“…Metastatic relapse occurs almost exclusively in children with HRPFs 5,9 ; however, there is no general consensus on which patients should be given adjuvant therapy because the risk of metastatic relapse is variable in the different categories of patients with HRPFs. For example, this risk The 3-year probability of DFS for patients with MD was 0.78 vs 0.98 for those without MD (P = .004).…”

Section: Discussionmentioning

confidence: 99%

“…For example, this risk The 3-year probability of DFS for patients with MD was 0.78 vs 0.98 for those without MD (P = .004). appears to be higher in patients with PLONI (with and without invasion at the resection margin) 5,18 or scleral involvement, 19,20 so virtually all groups use adjuvant therapy to reduce this risk. 2 Conversely, only 4% of children with isolated massive choroidal invasion present with a metastatic relapse even when no adjuvant therapy is given.…”

Section: Discussionmentioning

confidence: 99%

“…2,4 Most groups recommend adjuvant chemotherapy to decrease the risk of extraocular relapse in patients with HRPFs, but it is not 100% effective given that some patients relapse despite adjuvant therapy. 3,5 As a result, predicting the risk of metastatic relapse only by the presence of HRPFs is imperfect because there are lower-risk children who would be cured with enucleation alone rather than with unnecessary chemotherapy, and there are children with HRPFs who still relapse after conventional adjuvant therapy who constitute a very high-risk population currently not identifiable by pathologic criteria alone.…”

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confidence: 99%

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Minimal Disseminated Disease in Nonmetastatic Retinoblastoma With High-Risk Pathologic Features and Association With Disease-Free Survival

et al. 2016

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IMPORTANCE Fatal metastatic relapse may occur in children with retinoblastoma and high-risk pathologic features (HRPFs). Minimal dissemination (MD) may be an additional tool for risk estimation. The use of cone-rod homeobox (CRX) transcription factor messenger RNA for MD evaluation in metastatic retinoblastoma was previously reported, but no data in nonmetastatic cases with HRPFs are available. OBJECTIVES To evaluate whether MD is detectable in patients with nonmetastatic retinoblastoma and to assess its prognostic effect on disease-free survival (DFS). DESIGN, SETTING, AND PARTICIPANTS This single-institution cohort study of patients with nonmetastatic retinoblastoma and HRPFs used prospectively defined inclusion criteria and a sampling strategy to procure bone marrow (BM) and cerebrospinal fluid (CSF) samples from May 1, 2007, through October 31, 2013. Median follow-up was 38 months (range, 8-89 months). Survival analysis was closed in December 2015, and no further updates were made after that point. INTERVENTIONS The study evaluated CRX messenger RNA by quantitative polymerase chain reaction in BM and CSF at diagnosis and follow-up. In 14 patients, GD2 synthase was used instead of CRX for CSF evaluation. Patients were treated under uniform guidelines. MAIN OUTCOMES AND MEASURES Metastatic relapse. RESULTS The study included 96 children (median age at study inclusion, 26 months; range, 1-168 months; 46 male [47.9%]; 50 female [52.1%]) with nonmetastatic retinoblastoma and HRPFs (isolated massive choroidal invasion in 14, postlaminar optic nerve invasion in 51 [26 with concomitant massive choroidal and 13 with scleral invasion], 12 with scleral invasion without postlaminar optic nerve invasion, and 7 with tumor at the resection margin of the optic nerve) were evaluated at the time of primary or secondary enucleation. Minimal dissemination was detected in 9 patients (7 BM samples and 2 CSF samples) and was associated with extension beyond the resection margin of the optic nerve and scleral involvement, but only the former was independently associated (adjusted odds ratio, 57.0; 95% CI, 4.8-678.2; P = .001). In addition, MD occurred in 8 of the 43 International Intraocular Retinoblastoma Classification group E eyes with glaucoma (18.6%) and in 8 of 80 (10%) and 1 of 16 children (6.3%) who underwent primary or secondary enucleation, respectively. Children with MD had a 3-year DFS of 0.78 compared with 0.98 in those without MD (95% CI for the difference in DFS, 0.17-0.23; P = .004). CONCLUSIONS AND RELEVANCE These findings identified a high-risk population of children with retinoblastoma and HRPFs with MD. Because the number of events was small, these results, which suggest that children with International Intraocular Retinoblastoma Classification group E retinoblastoma and glaucoma have a higher risk of MD at diagnosis, should not be considered definitive at this time.

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“…There are clinical features present at diagnosis which have been correlated with postlaminar optic nerve invasion, including buphthalmos [3], neovascularization of the iris, and secondary glaucoma [4]. Secondary glaucoma may also be predictive of massive choroidal invasion [5]. The International Intraocular Retinoblastoma Classification of Retinoblastoma (IIRC) was intended to predict success of systemic chemotherapy treatment, and Group D and E are the most advanced eyes in the classification system [6,7].…”

Section: Introductionmentioning

confidence: 99%

Optic Nerve Obscuration in Retinoblastoma: A Risk Factor for Optic Nerve Invasion

et al. 2017

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Background: The objective of this study is to evaluate the risk of optic nerve invasion associated with optic nerve obscuration at diagnosis or persisting during treatment. Methods: Retrospective review from 2011-2016 of patients with advanced retinoblastoma (Group D/E) with complete obscuration of the nerve at diagnosis and a second group of patients with persistent, complete obscuration throughout treatment. Results: Advanced retinoblastoma was diagnosed in 102 eyes of 86 patients. The optic nerve was obscured in 69 eyes (68%) at diagnosis. Of these, 30 (43%) underwent salvage therapy and 39 (57%) primary enucleation. Histopathologic analysis of primarily enucleated eyes showed 41% prelaminar and 15% postlaminar invasion. Four eyes in the salvage group demonstrated persistent nerve obscuration; 2 were subsequently enucleated without evidence of nerve invasion. Average follow-up was 23.5 months (range 1-62 months). Conclusions and Relevance: Optic nerve obscuration at diagnosis may be associated with postlaminar optic nerve invasion. While persistent, complete obscuration of the optic nerve by retinoblastoma during treatment is a poor prognostic sign for both globe salvage and vision, it does not appear, in this small cohort, to increase the risk of optic nerve invasion. With appropriate control of the intraocular tumor, these eyes can be salvaged.

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Choroidal Infiltration by Retinoblastoma: Predictive Clinical Features and Outcome

Cited by 6 publications

References 25 publications

Treatment of Nonmetastatic Unilateral Retinoblastoma in Children

Treatment of Nonmetastatic Unilateral Retinoblastoma in Children

Minimal Disseminated Disease in Nonmetastatic Retinoblastoma With High-Risk Pathologic Features and Association With Disease-Free Survival

Optic Nerve Obscuration in Retinoblastoma: A Risk Factor for Optic Nerve Invasion

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