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Purpose: The purpose of this study is to report on the efficacy and safety of topical chemotherapy alone for giant ocular surface squamous neoplasia (OSSN). Methods: In this retrospective, interventional series, 10 eyes with giant OSSN underwent exfoliative biopsy to confirm the diagnosis followed by application of interferon alpha 2b (IFN α2b) and/or 5 fluorouracil, 1% (5FU). Reported outcome measures were tumor response, visual acuity, recurrence, systemic metastasis, and treatment complications. Results: Ten patients (3 female, 7 male) had a mean age of 73 (median, 69; range 40–89) years. Mean tumor diameter was 13.1 (median, 12.3; range 8.2–19.4) mm. Five (50%) eyes were treated with IFN-α2b alone; 1 (10%) with 5-FU alone and 4 (40%) required both IFN-α2b and 5-FU. The mean duration of treatment was 3, 0.5, and 6.4 months for IFN-α2b alone, 5-FU alone, and both IFN-α2b and 5-FU respectively. Complete tumor response was observed in all 10 cases at mean follow-up of 12.8 (median, 11.5; range, 3–25) months. Complications noted were transient irritation and burning (n = 4), dry eyes (n = 2), and transient flu-like symptoms (n = 2). There was no evidence of chemotherapy-related symblepharon, stem cell deficiency, scleral thinning, or corneal opacity. There were no tumor recurrences, and no patient required surgical excision or cryotherapy. Conclusion: Topical chemotherapy was a safe and effective treatment, inducing complete regression in all cases of giant OSSN in this series. There were no sight-limiting complications.

Vitreoretinal lymphoma (VRL) is an ultra-rare disease and an unusual presentation of primary central nervous system lymphoma (PCNSL) [1]. A quarter of PCNSL patients develop VRL and upto 85% of VRL...

Purpose To evaluate the patterns of regression of iris melanoma after treatment with palladium-103 ( 103 Pd) plaque brachytherapy. Design Retrospective, nonrandomized, interventional case series. Participants Fifty patients with primary malignant melanoma of the iris. Methods Palladium-103 plaque brachytherapy. Main Outcome Measures Changes in tumor size, pigmentation, and vascularity; incidence of iris neovascularization; and radiation-related complications. Results The mean age in the case series was 61.2±14.9 years. The mean tumor thickness was 1.4±0.6 mm. According to the American Joint Committee on Cancer, eighth edition, staging criteria for iris melanoma, 21 tumors (42%) were T1a, 5 tumors (10%) were T1b, and 24 tumors (48%) were T2a. The tumor was melanotic in 37 cases (74%) and amelanotic in 13 cases (26%); of these, 13 tumors (26%) showed variable pigmentation. After brachytherapy, mean tumor thickness decreased to 0.9±0.2 mm. Pigmentation increased in 32 tumors (64%), decreased in 11 tumors (22%), and was unchanged in 6 tumors (12%). For intrinsic vascularity (n = 19), 12 tumors (63%) showed decrease and 7 tumors (37%) showed complete resolution. Appearance of ectropion uveae showed diminution in 15 tumors (43%); newly present corectopia was observed in 6 patients (12%). On high-frequency ultrasound imaging, of the 42 tumors (84%) with low to moderate internal reflectivity, 30 tumors (60%) showed an increase in internal reflectivity on regression. Iris stromal atrophy was noted in 26 patients (52%), progression or new-onset cataract was noted in 22 patients (44%), neovascular glaucoma was noted in 1 patient (2%), and there were no cases of corneal opacity. There was no clinical evidence (0%) of radiation-induced retinopathy, maculopathy, or optic neuropathy. Mean follow-up in this series was 5.2 years (range, 0.5–17 years). Conclusions The most common findings related to iris melanoma regression after 103 Pd plaque brachytherapy included decreased intrinsic tumor vascularity, increased tumor pigmentation, and decreased tumor thickness with synchronous increase in internal ultrasonographic reflectivity. No irreversible sight-limiting complications were noted.

Background/aims To evaluate the effect of adjuvant intravitreal triamcinolone acetonide (ITA) for radiation maculopathy (RM) recalcitrant to high-dose bevacizumab in patients with choroidal melanoma after plaque radiotherapy. Methods Eight eyes of eight patients with RM secondary to plaque radiotherapy for choroidal melanoma, recalcitrant to high-dose bevacizumab (3.0 mg) were retrospectively evaluated. Intravitreal injections of ITA (4 mg/0.1 mL) were performed at 4-week to 16-week intervals as an adjunct to continued bevacizumab therapy. Change in central foveal thickness (CFT) as measured by optical coherence tomography and change in visual acuity (VA) were the main outcome measures. Results At the time of diagnosis of choroidal melanoma, VA was 20/20 to 20/50 in 88% (n=7) and 20/60 to 20/200 in 12% (n=1). The mean radiation dose to the fovea was 81 Gy (median 75.2 Gy; range: 22.72–132.8 Gy). The mean onset to RM was 25 months after plaque therapy (median 25 months; range 12–44 months). At the time of initiation of ITA, VA was 20/20 to 20/50 in 38% (3/8), and 20/60 to 20/200 in 62% (5/8). After initiation of ITA, VA was stable or improved in 100% of patients (n=8) at 3 months, 88% at 6 months, 88% at 9 months and 75% at 12 months. Mean CFT was 417 µm at ITA initiation, 339 µm at 1 month, 355 µm at 6 months, 339 µm at 9 months and 359 µm at 1 year. Conclusion Intravitreal triamcinolone can be added to preserve vision and decrease macular oedema in patients with RM recalcitrant to high-dose anti-vascular endothelial growth factor agents.

The purpose of this study was to report on a case of Terson's syndrome (TS) using a novel instrument and technique to harvest posterior pole pathology from a postmortem eye. A modified ocular clamp was used to remove the posterior pole from the postmortem enucleated eye. Gross photographs were taken and an ocular sample of the posterior pole was sent to The New York Eye and Ear Pathology Laboratory. TS was identified from gross pathology and histologic examinations. The case history was consistent with that diagnosis. The authors concluded that high-quality gross and histopathologic examination of the posterior pole can be obtained with this novel instrument and technique. [ Ophthalmic Surg Lasers Imaging Retina. 2017;48:170–174.]

PURPOSE To report a case of neovascular and angle closure glaucoma secondary to breast cancer metastatic to the iris that was successfully treated with injections of intravitreal bevacizumab (Avastin) 1.25 mg/0.05 mL. METHODS Case report. PATIENTS A 47-year-old woman with metastatic breast cancer presented to The New York Eye Cancer Center with left ocular pain, photosensitivity, vision loss, and multiple iris nodules. Her intraocular pressure was uncontrolled. Gonioscopy revealed neovascularization of the iris and angle; no choroidal neovascularization was noted. Ultrasound biomicroscopy demonstrated tumor invasion of iris stroma with marked anterior uveal thickening and narrowed angles. RESULTS Three monthly injections of intravitreal bevacizumab resulted in nearly complete resolution of iris neovascularization, reduction of intraocular pressure, and control of tumor (although a small amount of residual tumor remained). CONCLUSION Intravitreal anti-vascular endothelial growth factor therapy for breast cancer metastatic to the iris with secondary neovascular glaucoma provided good local control for a limited follow-up period, because the patient died because of systemic complications of her disease.

Purpose To present a unique approach to eye and vision sparing for a patient with squamous cell carcinoma invading through a penetrating keratoplasty (PK) wound. Methods A 56-year-old functionally monocular man was found to have squamous cell carcinoma with intraocular invasion through a PK wound. Eye- and vision-salvaging palladium-103 plaque radiation was performed. An amniotic membrane graft was used to buffer and thus protect the cornea during the 7-day treatment. Leaded eye glasses were worn during the day to block emitted radiation and allow functional vision during irradiation. Results At 3.5 years status posttreatment, the tumor has regressed, PK has become progressively hazy (within the irradiated zone), and his vision is stable at 20/320. The epicorneal tumor has disappeared, and the intraocular tumor nodules are more highly reflective, but persistent. There has been no intraocular tumor progression, worsening of his glaucoma, or evidence of intraocular radiation vasculopathy. Conclusions Transcorneal intraocular tumor invasion was controlled using palladium-103 ophthalmic plaque radiation therapy applied through the natural and donor cornea. Definitive treatment resulted in local tumor control and preservation of vision.

Retinal capillary hemangiomas are benign vascular tumors of the retina. They can be unifocal, multifocal, unilateral or bilateral, sporadic or inherited. It is heritable as autosomal dominant inheritance when associated with von Hippel-Lindau (VHL) disease. Treatment of retinal capillary hemangioma can be observation, laser photocoagulation, cryotherapy and/or plaque radiotherapy. VHL disease can be associated with central nervous system hemangioma, renal cell carcinoma and visceral tumors. VHL gene is present on chromosome 3p25-26 and suspected individuals can therefore undergo genetic testing and focused systemic surveillance. This chapter describes the ophthalmic and systemic manifestations associated with retinal capillary hemangioma.

Counselling a patient and family members about choroidal melanoma is an essential arm of management. The diagnosis, survey for metastatic disease, staging, evidence-based treatment options, local with systemic outcomes and subsequent follow up visits need to be discussed. This chapter outlines the diagnosis, choice of treatment, special considerations, recommended systemic surveys and role of genetic counselling.

The emergence of technology has transformed ophthalmic oncology from a surgical specialty to one that involves both medical and radiation oncology. With that transition ocular oncologists began to use chemotherapeutic agents and specialized radiation techniques. The first use of radiation focused on eye and vision-sparing external beam for retinoblastoma and implant “plaque” brachytherapy for uveal melanoma. However, the evolution did not stop there. Laser photocoagulation was found useful in the treatment of radiation vasculopathy, cryotherapy for conjunctival tumors, topical chemotherapy, and more. Herein, we discuss the most recent additions to the armamentarium used by ocular oncologists to save eyes, vision, and life.

Circumscribed choroidal hemangioma is a benign vascular hamartoma without systemic associations. For symptomatic cases, treatment options are photodynamic therapy (PDT), transpupillary thermotherapy (TTT), intravitreal injection of anti-vascular endothelial growth factor (VEGF), or radiation therapy. CyberKnife radiosurgery is an image-guided radiation therapy that delivers radiation to lesions anywhere in the body with an accuracy of 0.5 mm without damage to surrounding structures. We report a case of circumscribed choroidal hemangioma which was successfully treated with CyberKnife therapy. The literature search reveals cases of uveal melanoma, intraocular, and periocular lymphoma successfully treated with CyberKnife therapy. To the best of our knowledge, this is the first report on such treatment for choroidal hemangioma.

Purpose Cross-characterization of retinal astrocytomas (RA) using ocular diagnostic imaging techniques. Methods Fundus photography, fluorescein angiography, optical coherence tomography, fundus autofluorescence images, and ultrasonography were used to define characteristics of RA. Results Thirteen patients had sporadic RA and 12 had tuberous sclerosis. Of the latter, 1 patient had multiple (n = 14), bilateral tumors and was excluded so as not to skew the analysis. Of those patients with a solitary RA, 63% were juxtapapillary. All were white to yellow. A mulberry-shaped surface was observed in 33%. Fluorescein angiography revealed intrinsic tumor vessels and late diffuse tumor staining in 42%. Optical coherence tomography revealed cells spreading from the tumor surface into the vitreous (75%), intraretinal fluid (25%), and multiple, ovoid hypolucencies within the tumor stroma (54%). Fundus autofluorescent imaging revealed that hypolucencies were hyperautofluorescent and solid tumor stroma appeared hypoautofluorescent. B-scan imaging revealed moderate to high internal tumor reflectivity (100%) with orbital shadowing (46%). Optical coherence tomography revealed a mean tumor thickness of 0.8 mm. Ultrasound imaging showed a mean thickness of 1.4 mm (range 1.0–1.7). Of the 21 patients subsequently followed in our centers, no tumor growth was noted (mean 54 months, range 1–96). Conclusions This study reveals unique characteristics of RA and suggests that RA were most commonly amelanotic, moderate to high echoic, ≤1.7 mm in thickness, near the optic disc, and nonprogressive. When observed, a mulberry-shaped surface was pathognomonic.

Herein we present a generalized algorithm for processing activity images. Existing algorithms are applicable only to particular cases, and a new, broader approach is therefore proposed here that is based on a set of tuneable filters for images, using an extension of the addition and subtraction operations. The choice of tuning parameter is determined based on the desired result in each experimental case. We include several objective criteria to guide the choice of the most suitable value for the parameter. The use of our approach is exemplified with typical images with dynamic speckle, such as reading under paper, corn seeds and ultrasound images. These examples are also potentials applications in engineering environments.

Ocular cancers are unique among the diseases of the eye, threatening both vision and life. In most cases, the diagnosis can be made utilizing a careful clinical history and specialized ocular examination. Eye cancer diagnosis relies heavily on imaging techniques such as high-frequency ultrasound, fluorescein angiography, anterior and posterior segment optical coherence tomography, computed tomography (CT), and magnetic resonance imaging (MRI). Once the diagnosis is established, treatment decisions depend on the tumor’s location, size, local extension, patterns of growth, and secondary complications. Treatment options include observation, local resection, chemotherapy (topical, intravenous, intra-arterial, or intravitreal), and radiation (ophthalmic plaque or external beam). Enucleation or exenteration is only employed if these eye- and vision-sparing treatments are not possible. The core of this comprehensive review is a consecutive series of the most common ocular tumor of each structure of the eye, anterior to posterior, including basal cell carcinoma of the eyelid, squamous conjunctival neoplasia, choroidal melanoma, retinoblastoma, ocular adnexal lymphoma, and metastatic orbital tumors.

Purpose To investigate and characterize the clinical features of subtype-specific orbital lymphoma. Design Retrospective, interventional case series. Methods The study included 7 international eye cancer centers. Patient data were collected from January 1, 1980 through December 31, 2017. A total of 797 patients with a histologically verified orbital lymphoma were included. The primary endpoints were overall survival, disease-specific survival, and progression-free survival. Results The median age was 64 years, and 51% of patients (n = 407) were male. The majority of lymphomas were of B-cell origin (98%, n = 779). Extranodal marginal zone B-cell lymphoma (EMZL) was the most frequent subtype (57%, n = 452), followed by diffuse large B-cell lymphoma (DLBCL) (15%, n = 118), follicular lymphoma (FL) (11%, n = 91), and mantle cell lymphoma (MCL) (8%, n = 66). Localized Ann Arbor stage IE EMZL and FL were frequently treated with external beam radiation therapy. DLBCL, MCL, and disseminated EMZL and FL were primarily treated with chemotherapy. EMZL and FL patients had a markedly better prognosis (10-year disease-specific survival of 92% and 71%, respectively) than DLBCL and MCL patients (10-year disease-specific survival of 41% and 32%, respectively). Conclusions Four lymphoma subtypes were primarily found in patients with orbital lymphoma: EMZL, DLBCL, FL, and MCL. The histologic subtype was found to be the main predictor for outcome, with EMZL and FL patients having a markedly better prognosis than DLBCL and MCL.

A black teenager presented with long-standing vision loss in his right eye. Clinical exam revealed increased conjunctival vascularity and total exudative retinal detachment of the right eye. Ultrasound imaging demonstrated a choroidal mass with moderate-to-high reflectivity consistent with a choroidal hemangioma. A closer external examination demonstrated a subtle reddish hue of the eyelids and cheek, consistent with a nevus flammeus. MRI of the brain revealed a T2 enhancing vascular mass. Collectively, these findings were consistent with Sturge-Weber Syndrome. This case highlights the need for a high degree of suspicion for Sturge-Weber Syndrome in dark-skinned individuals that can otherwise mask the nevus flammeus. Physical exam findings and radiographic evidence can be used to guide the diagnosis. [ Ophthalmic Surg Lasers Imaging Retina . 2019;50:183–186.]

Purpose The purpose of this study was to evaluate super-thick amniotic membrane grafts (ST-AMGs) for ocular surface reconstruction. Design Retrospective, interventional case series. Methods This was a single-center study of clinical practice that included select patients with typically large ocular surface abnormalities that required reconstruction. The intervention studied was surgical insertion of a ST-AMG for reconstruction or repair of the ocular surface. Main outcome measures included intraoperative handling, graft position at 1 week post implantation, graft dissolution at 3 weeks, epithelialization of the ocular surface and symblepharon. Results Eleven ST-AMGs were implanted after resection with cryotherapy: 5 conjunctival melanoma, 4 squamous cell carcinoma, 1 sebaceous carcinoma, and 1 atypical pterygium. In addition, 1 was implanted for scleral necrosis. ST-AMGs were up to nine times thicker than standard amniotic grafts and were therefore amenable to both running and interrupted 7-0 Vicryl sutures without cheese-wiring. All cases had a well-positioned ST-AMG at 1 week and 75% (n = 9) had partial graft dissolution at 3 weeks. Complete epithelialization without wound dehiscence was noted in all cases. However, secondary (after additional tumor treatment) symblepharon formed in 16.7% (n = 2). In all cases, the mean visual acuity and intraocular pressures remained unchanged during conjunctival reconstruction and subsequent secondary treatments. Post epithelialization adjuvant topical chemotherapy was given to extend treatment margins and treat presumed occult disease in 50% (n = 6). At mean follow-up of 25.5 months (median 10, range 3-90), 10 cases (83.3%) showed complete local tumor control, 1 showed revascularization of the scleral melt, and 1 required orbital exenteration. Conclusion ST-AMGs were easy to suture and relatively persistent. Epithelialization of the ocular surface without primary symblepharon formation was noted. ST-AMGs should be considered an alternative for ocular surface reconstruction.

Purpose: To provide a clinical description of a case of bilateral choroidal melanoma in a patient treated with palladium-103 (103Pd) plaque brachytherapy. Methods: An 81-year-old man presented with choroidal melanoma in both eyes. In consideration of tumor sizes and locations, treatment involved insertion of 103Pd radioactive plaque in the right eye followed by the left at an interval of 2 months. Results: At 1-year follow-up, bilateral local control has been associated with apical tumor height regression, and visual acuity has been preserved (20/20 in the right eye and 20/25 in the left). Exudative subretinal fluid has resolved. There has been no metastasis. Conclusion: Bilateral choroidal melanomas are rare, reported to be either simultaneous or sequential as well as treatable. In this case, we were able to achieve tumor regression and preserve visual acuity in both eyes after plaque brachytherapy.

PURPOSE To describe an unusual case of pediatric iris melanocytoma with pigment dispersion glaucoma that resolved after resection of the primary tumor. METHODS Retrospective case review of the clinical record, ultrasonographic images, and histopathology. RESULTS A 6-year-old Asian girl, with a dark iris tumor, pigment dispersion, and secondary glaucoma, was initially treated with topical antiglaucoma medication and observation. Rapid growth prompted biopsy, revealing melanocytoma. As the tumor continued to grow, excision of the primary tumor was performed. Surgery proved curative in that the pigment dispersion slowly reabsorbed and her glaucoma resolved. CONCLUSIONS In this case, rapid growth did not indicate malignant transformation. Initial observation for growth and judiciously timed surgical intervention prevented progression, loss of vision, and potentially the loss of the eye.

Purpose To present our 12-year experience with low-energy-photon, slotted eye plaque radiation therapy. Design Retrospective interventional case series. Methods Setting: The New York Eye Cancer Center. Study Population: Fifty-two consecutive patients with uveal melanomas near, touching, or surrounding the optic disc. Intervention: Slotted eye plaque radiation therapy. Main Outcome Measures: Change in visual acuity, local tumor control, radiation side effects, eye salvage, and systemic metastases. Results Tumors were peripapillary within 1.5 mm of the optic disc (n = 8, 15%), juxtapapillary touching ≤180 degrees (n = 23, 44%), or circumpapillary >180 degrees and encircling the disc (n = 21, 41%). Mean follow-up was 47 months (median 34 months, range 6-146 months). Radiation induced a mean 41.2% reduction in tumor thickness. Life table analysis showed that 69% of patients retained their visual acuities ≥ 20/40 and had a vision loss–free survival 84 months after treatment. Also, 90% of patients retained their visual acuity between 20/50 and 20/200 and had a vision loss–free survival 36 months after treatment. Slotted plaque brachytherapy was associated with 4% secondary cataract, 11% neovascular glaucoma, and no dry eye or eyelash loss. Local tumor control (no recurrence) was achieved in 98.1% of patients. Life table analysis showed an overall enucleation-free survival of 93% and metastasis-free survival of 94%. Conclusions Slotted plaque radiation therapy provided a normalized plaque-tumor position, such that the entire choroidal melanoma plus a 2- to 3-mm free margin of normal-appearing tissue was included in the targeted zone. At 12 years, slotted plaque radiation therapy resulted in high rates of local tumor control and vision and eye retention.

Purpose: To provide a clinical description of the long-term outcome of a 103Pd plaque-irradiated ciliary body melanoma with extrascleral extension while attempting to preserve a subadjacent glaucoma filtering bleb. Methods: A 75-year-old woman with pseudoexfoliative glaucoma for 17 years, 16 years status post argon laser trabeculoplasty, and 15 years status post trabeculectomy in the left eye, was diagnosed with an ipsilateral ciliary body melanoma with visible extrascleral extension. Treatment involved insertion of a 103Pd radioactive plaque over the functioning trabeculectomy, with removal 7 days later. At plaque insertion, amniotic membrane grafts were used to cover the plaque and protect the filtering site. Results: The tumor was successfully treated without clinical evidence of harm to the filtering bleb, with resultant stable intraocular pressure. However, the patient developed blebitis 1.5 years later. Though it resolved with topical antibiotic therapy, the bleb became less succulent. Two years postoperatively, she developed a spontaneous hyphema that resolved after one injection of transscleral bevacizumab 1.25 mg. Her tumor continually regressed in thickness. Without additional glaucoma surgery, her intraocular pressure remained well-controlled on topical medications for 6 years. Conclusions: Ciliary body melanoma with minimal extrascleral extension beneath a functioning filtering bleb can be treated using radioactive plaque therapy. In this case, we were able to achieve both tumor regression and glaucoma control by covering the plaque with an amniotic membrane graft.

PURPOSE To describe a patient with a presumed retinal pigment epithelium (RPE) tumor originating from unilateral RPE dysgenesis. METHODS Case report. RESULTS A 30-year-old woman with an unremarkable medical and ocular history was referred for an evaluation of progressive central metamorphopsia in her left eye. Visual acuity was 20/20 in her right eye and 20/25 in her left eye. Funduscopic examination of the left eye revealed an elevated mass within an area of unilateral RPE dysgenesis showing hyperpigmentation and hypopigmentation with scalloped margins. Fundus autofluorescence of the lesion showed a marginal pattern of hyperautofluorescence and hypoautofluorescence that was the inverse of the fluorescein angiography pattern. A well-circumscribed subretinal mass appeared to originate from the unilateral RPE dysgenesis lesion with surrounding subretinal fluid extending beneath the fovea. Ultrasonography showed medium-to-high reflectivity of the mass with no evidence of choroidal involvement. Optical coherence tomography showed a subretinal hyporeflective mass consistent with a tumor of RPE origin. The tumor appeared to invade the overlying retina where fluorescein angiography showed hyperfluorescent leakage and OCT angiography showed retinal vascular deformation. Indocyanine green angiography showed no evidence of choroidal neovascularization. Findings in the right fundus were normal. Over a 5-month follow-up, intravitreal anti-vascular endothelial growth factor therapy induced a resolution of subretinal exudation and modest reduction in tumor thickness. CONCLUSION To the authors' knowledge, this is the first report of a presumed RPE tumor described as originating from unilateral RPE dysgenesis. Multimodal imaging was crucial for establishing the diagnosis and showing that the patient's visual symptoms were a product of the exudation produced by the tumor's invasion of the retina.

Objective To evaluate extraocular muscle surgery associated with plaque brachytherapy for choroidal melanoma. Design Single-center retrospective cohort study. Participants Three hundred twenty-nine eyes of 329 consecutive patients with clinically diagnosed choroidal melanoma. Intervention Palladium 103 plaque brachytherapy with or without extraocular muscle surgery. Main Outcome Measures Type of muscle surgery required for each tumor location, timing, incidence, and duration of diplopia, as well as treatment. Results Two hundred fifty-four patients (n = 254/329; 77.2%) required muscle surgery. One hundred seven patients (n = 107/329; 32.5%) required surgery on 2 or more muscles. Of 373 muscles repositioned, the lateral rectus muscle (n = 115/373; 30.8%) and inferior oblique muscle (n = 70/373; 18.7%) were the most common, correlating to intraocular tumor location ( P Conclusions Extraocular muscle surgery frequently is required for plaque brachytherapy. Although transient diplopia occurred in 11.2% of patients, persistent diplopia occurred in only 1.9% of patients and was treatable.

Objective This study was conducted to compare the relative, clinical intraocular dose distribution for palladium-103 (103Pd) versus iodine-125 (125I) ophthalmic plaque radiation therapy. Methods Preoperative comparative radiation dosimetry was performed to evaluate 319 consecutive uveal melanomas treated between 2006 and 2012. Results There were 68 (21.3 %) anterior (iris and/or ciliary body) and 251 (78.7 %) choroidal melanomas examined in this study. According to AJCC staging, 7th edition, 146 (45.8 %) were T1, 126 (39.5 %) T2, 40 (12.5 %) T3, and 7 (2.2 %) T4. All were prescribed an equivalent tumor-apex dose. When compared to 125I, 103Pd was associated with a mean 41.9 % lower radiation dose to the opposite eye wall (p

Purpose: The purpose of this study is to describe the clinical characteristics, multimodality imaging findings, and clinical course of iris varices. Methods: Retrospective, noncomparative, observational case series of 28 eyes of 26 patients with iris varices, diagnosed between 2007 and 2017, has been used. Results: The mean (±SD) age was 58.3 ± 12.5 years (median 57.5, range 37–81). Patients were 57.7% male and 27% hypertensive. Varices were bilateral in two patients. The mean and median visual acuities were both 20/20 (range 20/16–20/40). Intraocular pressures were 16 mmHg (10–23 mmHg). Secondary glaucoma did not occur. The inferotemporal iris quadrant was affected in 75%. A single varix was seen in 64% and 36% appeared multiple. Varix orientation was radial in 57% and circumferential in 21%. Combined radial and circumferential varix orientation was noted in 18%. One had independent radial and circumferential varices in separate quadrants. A single episcleral sentinel blood vessel directed to the varix was present in 36%. Ultrasound biomicroscopy (UBM) showed a slightly increased mean iris thickness of 0.8 mm and multiple echolucent iris stromal vascular channels. Iris angiography showed no leakage of dye. Managed by observation over a mean follow-up of 37.7 months (range, 3–129), 96.4% eyes were stable and one (3.6%) regressed. No corectopia, ectropion uveae, hyphema, or metachronous anterior segment benign or malignant tumors occurred. Conclusion: Iris varix is primarily located in the inferotemporal quadrant and not associated with dysmorphic pupillary findings, progression, secondary glaucoma, or malignancy. Iris varices were benign vasculopathies with no associated ocular or vision-related morbidity.

To evaluate the safety and tolerability and treatment efficacy of high-dose (2.0 mg) intravitreal ranibizumab for recalcitrant radiation retinopathy.A phase I to II open-label, nonrandomized prospective clinical trial was performed on 10 eyes of 10 patients with recalcitrant radiation retinopathy who were failing standard dose anti-vascular endothelial growth factor (VEGF) therapy. External beam or plaque brachytherapy-associated retinopathy was characterized by persistent macular edema or leakage on optical coherence tomography or fluorescein angiography. Intravitreal 2.0 mg ranibizumab was given monthly up to 12 months and monitored for tolerability and change in best-corrected visual acuity (BCVA), central foveal thickness, and clinical signs of radiation retinopathy.Seven patients completed the 1-year study and received all 12 injections; 3 withdrew from the study due to worsening retinopathy (1 after external beam, 2 following plaque). Treatment was well-tolerated with no severe adverse reactions. A total of 70% had stable (n = 3) or improved (n = 4) BCVA. Mean change in BCVA was +3.3 letters at 6 months and +0.7 letters at 1 year. Mean improvement in central foveal thickness (CFT) was -19.3% (range -57 to +15%) at 1 year. Initial mean CFT was 428 µm (range 192-776); final mean CFT was 333 µm (range 190-532). A total of 80% demonstrated a statistically significant (plt;0.05) reduction in CFT.Regardless of radiation source, intravitreal injections of 2.0 ranibizumab induced significant reductions in macular edema and maintained or improved BCVA in most patients who were failing standard dose anti-VEGF therapy.

Purpose To evaluate outcomes after ophthalmic plaque radiation therapy for small choroidal melanomas. Design Retrospective study, case series. Participants Seventy-two patients with choroidal melanomas ≥1.5 and ≤2.4 mm apical height and ≤10 mm width treated between 2002 and 2012, with a minimum follow-up of 8 months. Methods All patients were treated with palladium-103 plaque brachytherapy. Mean radiation dose to the tumor apex was 82.4 Gy (range, 70.0–102 Gy). Main Outcome Measures Local control, radiation complications, visual acuity, and metastatic rate. Results Plaque radiotherapy provided 100% local tumor control and eye retention at a mean 54 months of observation (95% confidence interval, 46–63 months). The most common long-term brachytherapy-related complications were radiation maculopathy (43.1%) and radiation optic neuropathy (20.8%) developing at a mean of 27 months (range, 9–72 months) after irradiation. In this series, 94.4% of patients maintained 20/200 or better vision. There has been no small choroidal melanoma–related metastasis. Conclusions Palladium-103 plaque radiation therapy offered excellent local control and visual acuity outcomes for patients with small choroidal melanoma. Financial Disclosure(s) The author(s) have no proprietary or commercial interest in any materials discussed in this article.

Purpose To present a case series of peripheral pigmented placoid corneal endotheliopathy (PPPCE). Methods A retrospective chart review of patient demographics, medical histories, and clinical characteristics was performed. Examinations included the following specialized imaging modalities: slit-lamp photography, gonioscopy, high-frequency ultrasound biomicroscopy, and anterior segment ocular coherence tomography. A PubMed and multiple corneal textbook literature search using the key words cornea, pigment, plaque, and endothelium revealed that no similar cases were reported. Results Five eyes in 4 asymptomatic female patients were affected. Their mean age was 53 years (range, 43-61 years), and 3 were of African American descent and 1 was of Hispanic descent. The PPPCE lesions had a vertical dimension of 0.2 to 1.7 mm and a horizontal dimension of 0.5 to 6.1 mm. All the PPPCE lesions were well demarcated, brown, and peripherally located on the inferior corneal endothelium. Clock-hour meridians extended from 4 to 7 o'clock, with the largest PPPCE lesion spanning 4.3 clock hours. Gonioscopy revealed distinct well-circumscribed brown-pigmented plaques adherent to the corneal endothelium with no extension beyond the trabecular meshwork. Ultrasound biomicroscopy and anterior segment ocular coherence tomography revealed the presence of hyperreflective lesions with no corneal stromal invasion, edema, or epitheliopathy. There were no synchronous anterior or posterior segment abnormalities. The PPPCE lesions have remained unchanged for a mean of 17 months (range, 8-34). Conclusions Four healthy patients were noted to have PPPCE lesions. Although their etiology remains unknown, PPPCE behavior, morphology, and inferior corneal location suggest an origin from iris stromal melanocytes or iris pigment epithelium.

BACKGROUND AND OBJECTIVE: To report results after amniotic membrane–protected epicorneal plaque brachytherapy for anterior uveal melanoma. PATIENTS AND METHODS: Sixty-three patients were treated with epicorneal radioactive plaques for 5 to 7 days. To prevent corneal damage, eye irritation, and reduce pain, 0.1-mm-thick fresh-frozen amniotic membrane grafts were interposed between the plaque and the cornea during brachytherapy. All amniotic membranes were removed at the end of brachytherapy. Patients were questioned about subjective comfort during treatment and observed for clinical outcomes. RESULTS: Only 4.8% of patients reported pain during brachytherapy. Short-term brachytherapy-related corneal complications including mild keratopathy, corneal epithelial defect, and edema were noted in 41.3% of patients. All corneal complications resolved within 2 weeks. No patients developed corneal melting or opacity during a mean follow-up of 28 months. Local tumor control was achieved in 95.2%. CONCLUSION: Amniotic membrane–buffered epicorneal plaque therapy provided comfort and excellent local tumor control. [ Ophthalmic Surg Lasers Imaging Retina . 2013;44:477–482.]

The diagnosis and treatment of orbital and periorbital malig- nancies are challenging. These tumors can result in blindness, death, and significant cosmetic deformities. Herein, we present the most common ocular malignancies and a systematic approach to diagnosis. Further, we integrate the seventh edition American Joint Committee on Cancer staging system, biomarkers, and multidisciplinary approaches to treatment.

To evaluate the prognostic utility of the American Joint Committee on Cancer (AJCC) staging system for ocular adnexal lymphoma (OAL). A multicenter, consecutive case series of patients with biopsy-proven conjunctival, orbit, eyelid, or lacrimal gland/sac lymphoma was performed. The electronic pathology and clinical records were reviewed for new or recurrent cases of ocular adnexal lymphoma. The main outcome measures included pathology and clinical staging (AJCC and Ann Arbor systems), treatment, and recurrence (local and systemic). Statistical analysis included demographic evaluations and the Kaplan-Meier survival probability method. Extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue were the most common (n=60/83, 72%). The most common Ann Arbor clinical stages were IE (76%) followed by IIE (17%) and IIIE (7%). Pathology identified 13 cases (15%) that were upstaged to group IV (p=0.017). Similarly, AJCC clinical stages were cT1NOMO (21.7%), cT2NOMO (44.6%), cT3N0M0 (5%), and cT4NOMO (2.4%). Local control was achieved in 75% of treated patients. There were 19 local recurrences from which 14 (74%) belonged to the non-radiation treatment groups. Lower-risk groups (T1 and T2 without lymph node involvement or metastatic disease of AJCC and IE of Ann Arbor) had longer disease-free survival than the higher-risk groups (AJCC T1, T2 with nodal involvement or metastatic disease, T3, and T4 as well as Ann Arbor II, III, and IV). The overall mean follow-up was 43.3 months (range 6-274). Regardless of stage, recurrence and disease-free survival were more closely related to treatment and histopathology rather than tumor size or site-specific location.

Purpose To document subtype-specific clinical features of lymphoma of the eyelid, and their effect on patient outcome. Design Retrospective observational case series. Methods Patient data were collected from 7 international eye cancer centers from January 1, 1980 through December 31, 2015. The cases included primary and secondary lymphomas affecting the eyelid. Overall survival, disease-specific survival (DSS), and progression-free survival were the primary endpoints. Results Eighty-six patients were included. Mean age was 63 years and 47 (55%) were male. Non-Hodgkin B-cell lymphomas constituted 83% (n = 71) and T-cell lymphomas constituted 17% (n = 15). The most common subtypes were extranodal marginal-zone lymphoma (EMZL) (37% [n = 32]), follicular lymphoma (FL) (23% [n = 20]), diffuse large B-cell lymphoma (DLBCL) (10% [n = 9]), mantle cell lymphoma (MCL) (8% [n = 7]), and mycosis fungoides (MF) (9% [n = 8]). EMZL had a female predilection (69% [22 of 32]), whereas MCL (71% [5 of 7]) and MF (88% [7 of 8]) had a male predominance. MCL (57% [4 of 7]), DLBCL (56% [5 of 9]), and MF (88% [7 of 8]) were frequently secondary lymphomas. Localized EMZL and FL were mostly treated with external beam radiation therapy, whereas DLBCL, MCL, and high Ann Arbor stage EMZL and FL were frequently treated with chemotherapy. DLBCL and MCL had a poor prognosis (5-year DSS, 21% and 50%, respectively), whereas EMZL, FL, and MF had a good prognosis (5-year DSS, 88%, 88% and 86%, respectively). Conclusions Lymphoma of the eyelid consists mainly of the lymphoma subtypes EMZL, FL, DLBCL, MCL, and MF. High-grade DLBCL and MCL, as well as MF, are frequently secondary eyelid lymphomas. The main predictor of outcome was the histologic subtype: EMZL, FL, and MF had a significantly better prognosis than MCL and DLBCL.

Importance To date, the clinical features of the various subtypes of conjunctival lymphoma (CL) have not been previously evaluated in a large cohort. Objective To characterize subtype-specific clinical features of CL and their effect on patient outcome. Design, Setting, and Participants A retrospective multicenter study was performed. Patient data were collected from January 1, 1980, through December 31, 2010. The dates of the analysis were May 15, 2015, to August 20, 2015. The median follow-up period was 43 months. Seven eye cancer centers were involved in the study. In total, 268 patients with CL were identified, 5 of whom were excluded because of missing clinical data. Main Outcomes and Measures Overall survival, disease-specific survival, and progression-free survival were the primary end points. Results Two hundred sixty-three patients with CL were included in the study. Their mean age was 61.3 years, and 55.1% (145 of 263) were female. All lymphomas were of B-cell type. The most frequent subtype was extranodal marginal zone lymphoma (EMZL) (68.4% [180 of 263]), followed by follicular lymphoma (FL) (16.3% [43 of 263]), mantle cell lymphoma (MCL) (6.8% [18 of 263]), and diffuse large B-cell lymphoma (DLBCL) (4.6% [12 of 263). Conjunctival lymphoma commonly manifested in elderly individuals (age range, 60-70 years old), with EMZL having a female predilection (57.8% [104 of 180]) and MCL having a marked male predominance (77.8% [14 of 18]). Unlike EMZL and FL, DLBCL and MCL were frequently secondary diseases (41.7% [5 of 12] and 88.9% [16 of 18], respectively), with MCL showing a frequent occurrence of stage IVE lymphoma (61.1% [11 of 18]) and bilateral manifestation (77.8% [14 of 18]). Localized disease (stage IE or IIE) was commonly treated with external beam radiation therapy (EBRT) with or without chemotherapy, while widespread lymphoma (stage IIIE or IVE) and MCL of any stage were managed with chemotherapy with or without EBRT. Diffuse large B-cell lymphoma and MCL had a poor prognosis, with 5-year disease-specific survival of 55.0% and 9.0%, respectively, in contrast to EMZL (97.0%) and FL (82.0%). Further survival predictors included age (EMZL), sex (FL), and Ann Arbor staging classification (EMZL and FL). The American Joint Committee on Cancer TNM staging showed limited prognostic usefulness, only being able to predict survival for patients with DLBCL. Conclusions and Relevance Conjunctival lymphoma consists of mainly 4 subtypes of B-cell non-Hodgkin lymphoma: EMZL, FL, MCL, and DLBCL. Mantle cell lymphoma is characterized by a particularly high frequency of secondary disease of stage IVE and bilateral manifestation. The histological subtype is the main outcome predictor, with MCL and DLBCL having a markedly poorer prognosis than EMZL and FL.

Dosimetry of eye plaques for ocular tumors presents unique challenges in brachytherapy. The challenges in accurate dosimetry are in part related to the steep dose gradient in the tumor and critical structures that are within millimeters of radioactive sources. In most clinical applications, calculations of dose distributions around eye plaques assume a homogenous water medium and full scatter conditions. Recent Monte Carlo (MC)-based eye-plaque dosimetry simulations have demonstrated that the perturbation effects of heterogeneous materials in eye plaques, including the gold-alloy backing and Silastic insert, can be calculated with reasonable accuracy. Even additional levels of complexity introduced through the use of gold foil "seed-guides" and custom-designed plaques can be calculated accurately using modern MC techniques. Simulations accounting for the aforementioned complexities indicate dose discrepancies exceeding a factor of ten to selected critical structures compared to conventional dose calculations. Task Group 129 was formed to review the literature; re-examine the current dosimetry calculation formalism; and make recommendations for eye-plaque dosimetry, including evaluation of brachytherapy source dosimetry parameters and heterogeneity correction factors. A literature review identified modern assessments of dose calculations for Collaborative Ocular Melanoma Study (COMS) design plaques, including MC analyses and an intercomparison of treatment planning systems (TPS) detailing differences between homogeneous and heterogeneous plaque calculations using the American Association of Physicists in Medicine (AAPM) TG-43U1 brachytherapy dosimetry formalism and MC techniques. This review identified that a commonly used prescription dose of 85 Gy at 5 mm depth in homogeneous medium delivers about 75 Gy and 69 Gy at the same 5 mm depth for specific (125)I and (103)Pd sources, respectively, when accounting for COMS plaque heterogeneities. Thus, the adoption of heterogeneous dose calculation methods in clinical practice would result in dose differences >10% and warrant a careful evaluation of the corresponding changes in prescription doses. Doses to normal ocular structures vary with choice of radionuclide, plaque location, and prescription depth, such that further dosimetric evaluations of the adoption of MC-based dosimetry methods are needed. The AAPM and American Brachytherapy Society (ABS) recommend that clinical medical physicists should make concurrent estimates of heterogeneity-corrected delivered dose using the information in this report's tables to prepare for brachytherapy TPS that can account for material heterogeneities and for a transition to heterogeneity-corrected prescriptive goals. It is recommended that brachytherapy TPS vendors include material heterogeneity corrections in their systems and take steps to integrate planned plaque localization and image guidance. In the interim, before the availability of commercial MC-based brachytherapy TPS, it is recommended that clinical medical physicists use the line-source approximation in homogeneous water medium and the 2D AAPM TG-43U1 dosimetry formalism and brachytherapy source dosimetry parameter datasets for treatment planning calculations. Furthermore, this report includes quality management program recommendations for eye-plaque brachytherapy.