Purpose: To evaluate the ability of the 8th edition of the American Joint Committee on Cancer (AJCC) Cancer Staging Manual to estimate metastatic and mortality rates for children with retinoblastoma (RB).Design: International, multicenter, registry-based retrospective case series.Participants: A total of 2190 patients from 18 ophthalmic oncology centers from 13 countries over 6 continents.Methods: Patient-specific data fields for RB were designed and selected by subcommittee. All patients with RB with adequate records to allow tumor staging by the AJCC criteria and follow-up for metastatic disease were studied.Main Outcome Measures: Metastasis-related 5-and 10-year survival data after initial tumor staging were estimated with the KaplaneMeier method depending on AJCC clinical (cTNM) and pathological (pTNM) tumor, node, metastasis category and age, tumor laterality, and presence of heritable trait.Results: Of 2190 patients, the records of 2085 patients (95.2%) with 2905 eyes were complete. The median age at diagnosis was 17.0 months. A total of 1260 patients (65.4%) had unilateral RB. Among the 2085 patients, tumor categories were cT1a in 55 (2.6%), cT1b in 168 (8.1%), cT2a in 197 (9.4%), cT2b in 812 (38.9%), cT3 in 835 (40.0%), and cT4 in 18 (0.9%). Of these, 1397 eyes in 1353 patients (48.1%) were treated with enucleation. A total of 109 patients (5.2%) developed metastases and died. The median time (n ¼ 92) from diagnosis to metastasis was 9.50 months. The 5-year KaplaneMeier cumulative survival estimates by clinical tumor categories were 100% for category cT1a, 98% (95% confidence interval [CI], 97e99) for cT1b and cT2a, 96% (95% CI, 95e97) for cT2b, 89% (95% CI, 88e90) for cT3 tumors, and 45% (95% CI, 31e59) for cT4 tumors. Risk of metastasis increased with increasing cT (and pT) category (P < 0.001). Cox proportional hazards regression analysis confirmed a higher risk of metastasis in category cT3 (hazard rate [HR], 8.09; 95% CI, 2.55e25.70; P < 0.001) and cT4 (HR, 48.55; 95% CI, 12.86e183.27; P < 0.001) compared with category cT1. Age, tumor laterality, and presence of heritable traits did not influence the incidence of metastatic disease.Conclusions: Multicenter, international, internet-based data sharing facilitated analysis of the 8th edition AJCC RB Staging System for metastasis-related mortality and offered a proof of concept yielding quantitative, predictive estimates per category in a large, real-life, heterogeneous patient population with RB.

Purpose: To evaluate the ability of the American Joint Committee on Cancer (AJCC) 8th edition to predict local tumor control and globe salvage for children with retinoblastoma (RB).Design: International, multicenter, registry-based retrospective case series.Participants: A total of 2854 eyes of 2097 patients from 18 ophthalmic oncology centers from 13 countries over 6 continents.Methods: International, multicenter, registry-based data were pooled from patients enrolled between January 2001 and December 2013. All RB eyes with adequate records to allow tumor staging by the AJCC 8th edition criteria and follow-up to ascertain treatment outcomes were included.Main Outcome Measures: Globe-salvage rates were estimated by AJCC clinical (cTNMH) categories and tumor laterality. Local treatment failure was defined as use of enucleation or external beam radiation therapy (EBRT), with or without plaque brachytherapy or intra-arterial chemotherapy (IAC).Results: Unilateral RB occurred in 1340 eyes (47%). Among the 2854 eyes, tumor categories were cT1 to cT4 in 696 eyes (24%), 1334 eyes (47%), 802 eyes (28%), and 22 eyes (1%), respectively. Of these, 1275 eyes (45%) were salvaged, and 1179 eyes (41%) and 400 eyes (14%) underwent primary and secondary enucleation, respectively. The 2-and 5-year KaplaneMeier cumulative globe-salvage rates without the use of EBRT by cTNMH categories were 97% and 96% for category cT1a tumors, 94% and 88% for cT1b tumors, 68% and 60% for cT2a tumors, 66% and 57% for cT2b tumors, and 32% and 25% for cT3 tumors, respectively. Risk of local treatment failure increased with increasing cT category (P < 0.001). Cox proportional hazards regression analysis confirmed a higher risk of local treatment failure in categories cT1b (hazard ratio [HR], 3.5; P ¼ 0.004), cT2a (HR, 15.1; P < 0.001), cT2b (HR, 16.4; P < 0.001), and cT3 (HR, 45.0; P < 0.001) compared with category cT1a. Use of plaque brachytherapy and IAC improved local tumor control in categories cT1a (P ¼ 0.031) and cT1b (P < 0.001).Conclusions: Multicenter, international, internet-based data sharing validated the 8th edition AJCC RB staging to predict globe-salvage in a large, heterogeneous, real-world patient population with RB.

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Purpose: To study the change in intraocular pressure (IOP) and angle status after phacoemulsification in a cohort of primary angle closure hypertension (PACHT) patients. Setting: Tertiary Eye Care, India. Design: Prospective interventional case-series. Methods: Case-series of 18 eyes of 18 patients. Preoperative biometry including axial length, anterior chamber depth (ACD), lens thickness, and central corneal thickness were studied. Preoperative and postoperative IOP, number of topical glaucoma medications, angle swept source optical coherence tomography (SS-OCT) parameters of nasal and temporal angle opening distance, trabecular iris space area, scleral spur angle, lens vault and circumferential iridotrabecular contact (ITC) were studied and their correlations derived. Results: The mean preoperative IOP, 31±6 mm Hg, decreased to 14±1 mm Hg at 6-months postoperative period, P<0.001, a fall by 58±14%. The number of glaucoma medications reduced from 4(3-5) to 2(1-3), P<0.001. All SS-OCT studied parameters denoted significant angle widening postsurgery. The ITC% reduced from 52(16-100) to 19(0-97), P<0.001. The preoperative ITC showed moderate to strong correlation with all preoperative nasal and temporal angle parameters. It also showed moderate positive correlation with percentage fall in IOP at 1-month postoperative period. The preoperative ACD showed significant negative correlation with preoperative and postoperative ITC. Conclusions: PACHT patients benefit significantly from cataract surgery with marked angle widening, IOP reduction and a decrease in the number of glaucoma medications. The SS-OCT derived circumferential iridotrabecular contact index can be used as the single best parameter to indicate the preoperative angle status and predict postoperative change in IOP, as against the numerous single section measured angle parameters.

leiomyoma actin melanoma immunohistochemistry benign uvea a b s t r a c tIntraocular leiomyoma is a benign smooth muscle tumor. First recognized before the era of immunohistochemistry, uveal leiomyomas have been described in case reports and small case series. We add 3 new cases, for a total of 80. Of these, there were 29 men and 51 women. The mean and median ages were 35.8 and 30.5 years respectively, with a range of 8 to 80 years. Curiously, ciliary body tumors were more common in females, whereas iris and posterior choroidal leiomyomas were more prevalent in males. Infrequently associated with systemic fibroids, nuclear expression of sex steroid receptors was inconsistent. Iris and posterior choroidal leiomyoma were predominantly amelanotic, while 40% of ciliary body leiomyomas were brown. Two-thirds of the leiomyomas blocked transillumination partially or completely, a feature shared by uveal melanoma. In general, low-frequency ultrasound imaging reveals low to moderate internal reflectivity; however, highfrequency anterior uveal ultrasound was used to localize a leiomyoma as resident in the suprachoroidal space with an overlying layer of intact choroid. In the few cases examined by physiologic imaging, increased metabolic activity (typically associated with malignancy and inflammation) has been noted. We found that pigmented uveal leiomyomas can be clinically identical to melanoma. Therefore, histopathology with immunohistochemical staining for smooth muscle actin was the most reliable diagnostic method to differentiate pigmented uveal leiomyoma from melanoma. Treatment is governed by the clinical diagnosis, tumor size and location, as well as prognosis for vision and globe preservation.

Congenital retinal macrovessels (CRM) are rarely reported, aberrant, large branches of retinal vessels that can be both arteries and veins; however, they are more commonly veins that cross the horizontal raphe to either supply or drain the macula. In this brief report, the authors discuss optical coherence tomography angiography features of CRM in a 12-year-old male patient presenting with low visual acuity in the right eye since childhood. To the authors' knowledge, this is the first such report published in the literature. [Ophthalmic Surg Lasers Imaging Retina. 2016;47:972-973.].

To investigate vascular and morphologic optic disc changes after slotted plaque radiation therapy for choroidal melanoma involving the optic disc.Design: Retrospective cross-sectional study.Participants: Thirty-nine patients with choroidal melanoma involving the optic nerve. Methods: Each melanoma was treated with palladium-103 slotted plaque brachytherapy (incorporating and/or surrounding the optic nerve sheath) between 2005 and 2019. Imaging of the optic nerve before and after radiation allowed for documentation and evaluation of optic nerve pallor and cup-to-disc ratio (CDR) changes. Optical coherence tomography (OCT) CDR measurements and intraocular pressure (IOP) were recorded pretreatment and at follow-up. Of these patients, 22 also had OCT angiography (OCT-A) images with sufficient quality for evaluation of blood vessel density and length. Differences in cup-to-disc measurements were correlated with changes in OCT-A-measured vessel density and length.Results: Following slotted plaque radiation therapy, there was no significant increase in IOP or optic nerve pallor. OCT and colour photography revealed significant increases (both p < 0.001) in CDR from pretreatment to the last follow-up. Increased CDRs on OCT were significantly correlated to OCT-A-measured change in vessel length (p = 0.027). Similarly, increased CDR ratios on fundus photography were significantly correlated with OCT-A-measured change in vessel density (p = 0.043) and length (p = 0.019).Conclusion: Fundus photography and OCT measurements revealed increased optic disc cupping following slotted plaque radiation therapy. Cupping was associated with OCT-A evidence of synchronous progressive peripapillary vascular occlusion and attenuation. Therefore, slotted plaque radiationÀinduced peripapillary and papillary ischemia was associated with increased CDR ratios and optic disc cupping.

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