Factors affecting eye conservation and metastasis in posterior uveal melanomas.

PURPOSE: To determine the effect of patient and tumor features and different treatments on eye removal (enucleation or exenteration) and metastasis in posterior uveal melanoma (PUM). METHODS: Retrospective analysis. Patient age (≤60 vs >60 years), sex (female vs male), visual acuity (VA, ≤20/40 vs >20/40), largest tumor basal diameter (LTBD), tumor thickness, tumor stage according to American Joint Committee on Cancer (AJCC) 8th edition, ciliary body involvement, distance to optic disc (OD)/fovea (≤3 mm vs >3 mm), OD involvement, and histopathology were evaluated. Primary treatment options were transpupillary thermotherapy, plaque radiotherapy, Cyberknife radiosurgery, exoresection, and eye removal. Risk factors for primary eye removal were determined using logistic regression test and those for secondary eye removal and metastasis with Cox regression analysis. RESULTS: Of 387 cases, 153 (39.5%) underwent primary eye removal. Multivariable risk factors for primary eye removal included AJCC tumor stage (p = 0.001, OR:4.586; p < 0.001, OR:34.545; p < 0.001, OR:103.468 for stages T2, T3, and T4 vs stage T1, respectively), and VA≤20/40 (p = 0.014, OR:2.597). Multivariable risk factors for secondary eye removal were VA≤20/40 (p = 0.019, RR:2.817) and AJCC stage T3 vs T1 (p = 0.021, RR:2.666). Eye preservation rates in patients undergoing eye-conserving treatments were 80.3%, 69.6%, and 51.5% at 5, 10, and 15 years, respectively. Metastasis-free survival rates were 81.0%, 73.0%, and 56.7% at 5, 10, and 15 years, respectively. Multivariable risk factors for metastasis included eye removal as primary treatment (p = 0.005, RR:2.828) and mixed type histopathology (p < 0.001, RR:4.804). DISCUSSION: Early diagnosis is crucial for both eye preservation and survival in PUM. Increasing AJCC tumor stage and lower VA were risk factors for eye removal in this study. Mixed type histopathology and primary eye removal were risk factors for metastasis.

Surgical Approach in Intraocular Tumors

Surgery in intraocular tumors is done for excision/biopsy and the management of complications secondary to the treatment of these tumors. Excision/biopsy of intraocular tumors can be done via fine-needle aspiration biopsy (FNAB), transretinal biopsy (TRB), partial lamellar sclerouvectomy (PLSU), and endoresection. FNAB, TRB, and PLSU can be used in tumors that cannot be diagnosed by clinical examination and other ancillary testing methods. PLSU is employed in tumors involving the iridociliary region and choroid anterior to the equator. Excisional PLSU is performed for iridociliary and ciliary body tumors with less than 3 clock hours of iris and ciliary body involvement and choroidal tumors with a base diameter less than 15 mm. However, for biopsy, PLSU can be employed with any size tumor. Endoresection is a procedure whereby the intraocular tumor is excised using vitrectomy techniques. The rationale for performing endoresection is based on the fact that irradiated uveal melanomas may cause complications such as exudation, neovascular glaucoma, and intraocular pigment and tumor dissemination (toxic tumor syndrome), and removing the dead tumor tissue may contribute to better visual outcome. Endoresection is recommended 1-2 weeks after external radiotherapy. Pars plana vitrectomy is also used in the management of complications including vitreous hemorrhage, retinal detachment, and epiretinal membrane that can occur after treatment of posterior segment tumors using radiotherapy and transpupillary thermotherapy. It is important to make sure the intraocular tumor has been eradicated before embarking on such treatment.

Secondary endoresection for previously treated choroidal melanomas with a non-responsive course and persistent exudative retinal detachment.

AIM: To evaluate the results and complications of secondary endoresection via pars plana vitrectomy for choroidal melanoma and review the previously reported endoresection studies on the treatment of choroidal melanoma. METHODS: The medical records of 6 patients with choroidal melanoma who underwent secondary endoresection between March 2012 and March 2020 were retrospectively reviewed. The indications for secondary endoresection were progressive or recurrent tumor and severe exudative retinal detachment after previous treatment with plaque radiotherapy/Cyberknife radiosurgery/transpupillary thermotherapy (TTT). RESULTS: Before endoresection, 2 eyes had Iodine-125 plaque radiotherapy and TTT, 1 eye had Ruthenium-106 plaque radiotherapy and TTT, 1 eye had Cyberknife radiosurgery and TTT, 1 eye had Cyberknife radiosurgery, and 1 eye had TTT only. Preoperative visual acuity ranged from 20/63 to 20/1600 (Snellen) and from 0.5 to 1.9 (mean: 1.1) on the logMAR scale. The mean tumor base diameters were 9.5×8.7 mm and the mean tumor thickness was 5.4 mm. After secondary endoresection, transient vitreous hemorrhage developed in 2 (33.3%) eyes and retinal detachment in 1 (16.7%) eye. Cytopathological examination revealed epithelioid cell melanoma in 4 (66.7%) eyes and mixed cell melanoma in 1 (16.7%). Melanoma cell type was not specified in 1 (16.7%) eye. At a mean follow-up of 49.6mo (range: 16-90mo), mean visual acuity did not improve and 1 eye was enucleated due to tumor recurrence. Final visual acuity ranged from 20/63 to 20/1600 (Snellen) and from 0.5 to 1.9 (mean: 1.2) on the logMAR scale. Two patients with choroidal melanoma developed metastasis and eventually expired. CONCLUSION: Secondary endoresection seems to be an effective treatment option for globe salvage in choroidal melanoma not responsive to conventional treatment and displaying persistent exudative retinal detachment. There was no visual acuity increase among the treated eyes but globe salvage was possible in most cases in this study.

Recurrence and new tumor development after frontline intravenous chemotherapy for retinoblastoma: Risk factors and treatment results.

PURPOSE: To evaluate the risk factors leading to recurrence and new tumor (NT) development in patients with retinoblastoma after intravenous chemotherapy (IVC) and to review the treatment outcomes. MATERIALS AND METHODS: The records of 166 retinoblastoma cases (having 246 affected eyes) who underwent six-cycle IVC (vincristine, etoposide, and carboplatin) as primary treatment between October 1999 and August 2020 were reviewed retrospectively. RESULTS: The mean ages at presentation were 9.0 (median: 8.0) and 9.2 (median: 8.5) months in cases with recurrence and NTs respectively. Recurrence was detected in 40 (16.3%) eyes, NTs in 29 (11.8%), and both recurrence/NTs in 24 (9.8%). The mean time elapsed till recurrence and NT was 10.7 months. Multivariable analysis showed that the factors predictive of recurrence were largest tumor base diameter (LTBD) >12 mm (p = 0.039) and presence of subretinal seeds at diagnosis (p = 0.043). Multivariable risk factors for the development of NTs were bilateral familial retinoblastoma (p = 0.001) and presence of subretinal seeds at diagnosis (p = 0.010). Mean follow-up was 80.1 (median: 72.5) months. By Kaplan-Meier analysis, the 1-, 3-, and 6-year recurrence and NT rates were 21.2%, 28.1%, and 28.7% and 14.9%, 22.6%, and 23.9% respectively. The most common treatment methods used for recurrent and/or NTs included cryotherapy, transpupillary thermotherapy, and intra-arterial chemotherapy. Enucleation was eventually required in 24/93 (25.8%) eyes. No patient developed metastasis. DISCUSSION: Development of recurrence and/or NT after IVC was noted in 38% of all retinoblastoma eyes. Bilateral familial disease, LTBD >12 mm, and presence of subretinal seeds at baseline were risk factors for recurrence and NTs in this study.

Circumscribed choroidal hemangioma: Comparative efficacy of transpupillary thermotherapy, indocyanine green-enhanced transpupillary thermotherapy, and photodynamic therapy and analysis of baseline clinical features effecting treatment outcomes.

PURPOSE: To compare the efficacy of transpupillary thermotherapy (TTT), indocyanine green-enhanced TTT (ICG-TTT), and photodynamic therapy (PDT) in the management of circumscribed choroidal hemangioma (CCH) and to investigate the baseline clinical features effecting treatment outcomes METHODS: Retrospective review of clinical records of 60 eyes with CCH which underwent TTT (25), ICG-TTT (22), or PDT (13). Main study outcomes were 1) final visual acuity (VA) ≤20/200, 2) <2 Snellen lines VA increase, 3) persistent subretinal fluid (SRF), and 4) <20% decrease in tumor thickness. RESULTS: Multivariable factors associated with final VA ≤20/200 included longer duration of symptoms (p = 0.015), lower initial VA (p = 0.030), and presence of retinoschisis overlying the tumor (p = 0.047). Multivariable factors for <2 Snellen lines VA increase were longer duration of symptoms (p = 0.018) and previous failed treatment (p = 0.003). By multivariable analysis, the only significant factor for persistent SRF was the presence of retinoschisis (p = 0.001). Multivariable factors associated with a decrease in tumor thickness by <20% were smaller initial tumor thickness (p = 0.045) and presence of retinoschisis (p = 0.014). By Pearson Chi-Square/Fisher Exact Test, final VA ≤20/200 rates (48.0%, 13.6%, 30.8% respectively, p = 0.041) and VA improvement by <2 lines rates (64.0%, 27.3%, 38.5% respectively, p = 0.036) were significantly different between TTT, ICG-TTT, and PDT groups. However, by post hoc analysis, ICG-TTT yielded statistically better outcomes compared to TTT with respect to final visual acuity (VA) ≤20/200 (p = 0.012) and <2 Snellen lines VA increase (p = 0.012). CONCLUSIONS: Longer symptom duration, previous failed treatment, lower initial VA, and presence of retinoschisis were risk factors for worse visual outcomes. Eyes with retinoschisis displayed more SRF persistence after treatment. Smaller initial tumor thickness and presence of retinoschisis were risk factors for <20% decrease in tumor thickness.

A 20-year audit of retinoblastoma treatment outcomes.

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%.