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Tumors of the conjunctiva and cornea comprise a large and varied spectrum of conditions. These tumors are grouped into two major categories of congenital and acquired lesions. The acquired lesions are further subdivided based on origin of the mass into surface epithelial, melanocytic, vascular, fibrous, neural, histiocytic, myxoid, myogenic, lipomatous, lymphoid, leukemic, metastatic and secondary tumors. Melanocytic lesions include nevus, racial melanosis, primary acquired melanosis, melanoma, and other ocular surface conditions like ocular melanocytosis and secondary pigmentary deposition. The most frequent nonmelanocytic neoplastic lesions include squamous cell carcinoma and lymphoma, both of which have typical features appreciated on clinical examination. The caruncle displays a slightly different array of tumors compared to those elsewhere on the conjunctiva, as nevus and papilloma are most common, but oncocytoma and sebaceous gland hyperplasia, adenoma, and carcinoma can be found. In this report, we provide clinical description and illustration of the many conjunctival and corneal tumors and we discuss tumor management. [ABSTRACT FROM AUTHOR]

Melanocytoma is a deeply pigmented variant of melanocytic nevus that classically occurs in the optic disk, sometimes with contiguous involvement of the adjacent retina or choroid. Historically, this tumor was often confused with malignant melanoma both clinically and histopathologically. Today, however, it is generally recognized by its typical clinical features that differ from most melanomas and erroneous enucleation is rarely done. Histopathologically, melanocytoma is composed of intensely pigmented round to oval nevus cells with benign features. Although traditionally believed to be a relatively stationary lesion, it is now known to exhibit minor enlargement in 10--15% of cases and can cause minor visual loss by a variety of mechanisms. In rare instance, it can induce severe visual loss due to spontaneous necrosis of the lesion or compressive optic neuropathy. More importantly, it can exhibit malignant transformation into melanoma in 1--2% of cases. Ophthalmologists should be familiar with melanocytoma of the optic disk and affected patients should be followed periodically. [ABSTRACT FROM AUTHOR]

Artificial intelligence (AI) is now integrated into a variety of fields, including medicine. AI applications raise numerous ethical questions, particularly about quality of care and patientclinician relationships. This article accompanies 2 digital photopaintings that address these ideas narratively and visually, with special emphasis on communication, compassion, and competence. [ABSTRACT FROM AUTHOR]

Prior to the 1970s, there were no medical specialists in the world who dealt exclusively with the diagnosis and management of ocular tumors such as uveal melanoma, retinoblastoma, and other tumors of the eyelid, conjunctiva, and orbit. I was headed to a general ophthalmology practice but Dr Richard Green, a well-known pathologist, stimulated my interest in pathology and ocular oncology. Dr P. Robb McDonald, Director of the Retina Service, offered me a retina fellowship, and, later, Dr. William Annesley, suggested that my training was ideal for development of an Eye Tumor Service. [Extracted from the article]

An 8-year-old girl was referred for an amelanotic iris tumor believed to have enlarged slightly over 3 months, suspicious for juvenile xanthogranuloma (JXG). The affected right eye had a lightly pigmented, vascular iris mass measuring 6 mm in basal dimension and 2 mm in thickness. There were no feeder vessels, seeding, inflammatory cells, or cutaneous abnormalities. Diagnostic fine needle aspiration biopsy (FNAB) was performed, revealing spindle B melanoma cells that were immunoreactive for melanocytic markers HMB45 and Melan-A. Complete tumor resection by basal sector iridectomy was performed. Histopathology confirmed spindle B melanoma. At 14-years follow up, there has been no recurrence or metastasis and visual acuity remains 20/25. Iris melanoma can develop in children and clinically resemble nodular JXG. [ABSTRACT FROM AUTHOR]

Purpose To review the management of ciliary body and choroidal melanoma (posterior uveal melanoma [PUM]) over the last century with an emphasis on changing concepts. Design Retrospective review. Participants Review of personal experience over 40 years and pertinent literature on management of PUM. Methods Diagnosis and therapy for PUM. Main Outcome Measures Patient survival. Results In the early 1900s, most patients presented with a large symptomatic melanoma that necessitated enucleation, and the systemic prognosis was poor. In the 1970s, controversy erupted regarding the role of enucleation for PUM. Some authorities advocated prompt enucleation, and others proposed that enucleation promoted metastasis, known as the “Zimmerman hypothesis.” Others recommended observation, withholding treatment until tumor growth was documented. During the 1970s, there was a trend toward eye-saving procedures, including laser photocoagulation, surgical removal of tumor, and techniques of radiotherapy. Despite local treatment success, systemic prognosis remained guarded with approximately 40% mortality overall. However, there was convincing evidence that smaller tumors offered a significantly better prognosis. Currently, there is a movement toward earlier identification and treatment of small melanomas using clinical factors predictive of malignant potential, in keeping with similar philosophy regarding other cancers. Further understanding of melanoma cytogenetics and molecular pathways have helped to recognize patients at risk for metastasis. At-risk patients are offered systemic therapeutic trials to prevent metastasis. We anticipate that the future management of PUM will focus on detection of clinical and imaging clues for earliest diagnosis, prompt local tumor treatment, and systemic targeted therapies for microscopic metastasis or prevention of metastasis. Personalized evaluation of patient-specific melanoma molecular pathway signature could allow for therapeutic intervention at a site specific to the pathway abnormality that leads to the development of melanoma. Conclusions Management of PUM has made major strides over the past century from the days of enucleation for massive, fatal tumor to early detection of smallest tumors with a more favorable prognosis. Current and future targeted specific tumor pathway interruption using systemic agents could improve survival. [ABSTRACT FROM AUTHOR]

Objective: To report clinical and histopathologic features of vascular tumors of the conjunctiva. Design: Retrospective, noninterventional case series. Participants: A total of 140 patients. Intervention: None. Main Outcome Measures: Tumor diagnosis, anatomic location, clinical features, management, and histopathology. Results: There were 140 vascular tumors of the conjunctiva with 93% benign and 7% malignant. The specific diagnoses included lymphangioma/lymphangiectasis (n = 54, 36%), pyogenic granuloma (n = 31, 22%), capillary hemangioma (n = 20, 14%), Kaposi''s sarcoma (n = 10, 7%), acquired sessile hemangioma (n = 10, 7%), racemose hemangioma (n = 7, 5%), varix (n = 4, 3%), cavernous hemangioma (n = 3, 2%), and glomangioma (n = 1, <1%). The lesions were unilateral in 89% and discovered at median age of 41 years. Bilateral lesions included lymphangiectasia, Kaposi''s sarcoma, and racemose hemangioma. All tumors were typically found in adults with the exception of capillary hemangioma (<1 year) and glomangioma (16 years). The median tumor diameter was 6 mm, with the largest median diameter (15 mm) with Kaposi''s sarcoma and (12 mm) glomangioma and capillary hemangioma and the smallest median diameter (3 mm) with cavernous hemangioma. The most common anatomic sites included bulbar conjunctiva, which was extralimbal in 80 patients (57%), limbal in 19 patients (14%), tarsal conjunctiva in 27 patients (19%), and forniceal conjunctival in 24 patients (17%). Clinical features included cystic component in 24% of patients (seen with lymphangioma, glomangioma, and varix), well-defined margins in 64% of patients, and feeder vessels in 39% of patients. Conclusions: A variety of vascular tumors can occur in both children and adults at various anatomic locations in the conjunctiva. They tend to occur unilaterally on the bulbar conjunctiva, and the majority of tumors are benign. Financial Disclosure(s): The author(s) have no proprietary or commercial interest in any materials discussed in this article. [Copyright &y& Elsevier]

Conservative management of retinoblastoma has evolved from external beam radiotherapy to systemic chemotherapy by intravenous route and now to localized chemotherapy by intra-arterial route in some cases. With 16-year experience, systemic chemotherapy has been found effective for minimal to moderately advanced retinoblastoma with tumour control of 90% or better, few side effects and even hope for return of some vision. Localized intra-arterial chemotherapy with delivery under fluoroscopy and catheterization of the ophthalmic artery is now undergoing evaluation and appears to provide striking control for retinoblastoma, particularly recurrent tumour seeds following other therapies. The limitations and complications of this approach have yet to be defined. Toxicity of the chemotherapy to the delicate retinal vessels is unknown. Despite its allure, intra-arterial chemotherapy should be used with caution, as in other fields of paediatric oncology it has been found to provide no advantage over intravenous chemotherapy. Time will tell. [ABSTRACT FROM AUTHOR]

The article focuses on the clinical features, diagnosis and management of sebaceous adenocarcinoma of the eyelid. It notes that the condition has a tendency to manifest diffuse, invasive growth in the eyelid and conjunctiva, and can metastasize to regional lymph nodes and distant organs. Different ways to manage such a condition consisted of local map biopsies, local resection and advanced methods of reconstruction, combined with cryotherapy and topical chemotherapy.

Abstract: Primary ocular melanoma can involve the uveal tract, conjunctiva, eyelid, or orbit. Uveal melanoma is the most common ocular melanoma and carries a serious prognosis, especially if the tumor is medium or large in size. Conjunctival melanoma manifests on the surface of the eye and has been increasing in incidence. Eyelid and primary orbital melanoma are the least common variants. Early diagnosis from annual ocular examination by an experienced ophthalmologist and treatment strategies are reviewed. [Copyright &y& Elsevier]

Purpose: To evaluate the clinical features and risks for transformation of conjunctival primary acquired melanosis (PAM) into melanoma. Design: Noncomparative case series. Participants: Three hundred eleven eyes with conjunctival PAM without melanoma at initial examination from a single-center tertiary referral center. Methods: Retrospective chart review with evaluation of the clinical features of PAM at initial presentation and follow-up. Times to PAM enlargement, recurrence, and transformation into melanoma were assessed using Kaplan–Meier estimates. Risk factors for these outcomes were analyzed using Cox proportional hazards regressions. Main Outcome Measures: Primary acquired melanosis enlargement, recurrence, and transformation into melanoma. Results: Mean patient age at diagnosis of PAM was 56 years (range, 15–90), 62% were female, and 96% were Caucasian. The conjunctival quadrant(s) affected by PAM were temporal (57%), inferior (45%), nasal (42%), and superior (37%). The anatomic location(s) of PAM included bulbar conjunctiva (91%), limbal conjunctiva (55%), cornea (23%), forniceal conjunctiva (13%), palpebral conjunctiva (12%), and caruncle (11%). Primary acquired melanosis extended for a mean of 3 clock hours (range, 1–12). Initial management included observation (n = 194 eyes [62%]), biopsy combined with cryotherapy (n = 107 eyes [34%]), and topical chemotherapy and/or cryotherapy without biopsy (n = 10 [4%]). Of PAM that was observed, Kaplan–Meier estimates at 10 years revealed PAM enlargement in 35% and transformation into melanoma in 12%. Of those that underwent incisional or excisional biopsy, 10-year estimates of PAM recurrence and transformation into melanoma were 58% and 11%, respectively. Progression to melanoma occurred in 0% of cases of PAM without atypia, 0% of cases of PAM with mild atypia, and 13% of cases of PAM with severe atypia. Of the 9 patients with PAM who developed melanoma, none have developed systemic metastasis. Multivariable analysis revealed that the most significant factor for both PAM recurrence and progression to melanoma was extent of PAM in clock hours. Conclusion: Primary acquired melanosis without atypia or with mild atypia shows 0% progression to melanoma, whereas PAM with severe atypia shows progression to melanoma in 13%. The greater the extent of PAM in clock hours, the greater the risk for transformation to melanoma. [Copyright &y& Elsevier]

Abstract: Melanocytoma is a deeply pigmented variant of melanocytic nevus that classically occurs in the optic disk, sometimes with contiguous involvement of the adjacent retina or choroid. Historically, this tumor was often confused with malignant melanoma both clinically and histopathologically. Today, however, it is generally recognized by its typical clinical features that differ from most melanomas and erroneous enucleation is rarely done. Histopathologically, melanocytoma is composed of intensely pigmented round to oval nevus cells with benign features. Although traditionally believed to be a relatively stationary lesion, it is now known to exhibit minor enlargement in 10–15% of cases and can cause minor visual loss by a variety of mechanisms. In rare instance, it can induce severe visual loss due to spontaneous necrosis of the lesion or compressive optic neuropathy. More importantly, it can exhibit malignant transformation into melanoma in 1–2% of cases. Ophthalmologists should be familiar with melanocytoma of the optic disk and affected patients should be followed periodically. [Copyright &y& Elsevier]

Objective: To report the case of a 59-year-old man with a right lacrimal gland mass, subsequently diagnosed as primary ductal adenocarcinoma of the lacrimal gland. Design: Interventional case report. Methods: We report the clinical presentation, histopathologic and immunohistochemical features, and treatment of a patient with primary ductal adenocarcinoma of the lacrimal gland. The current literature regarding this uncommon neoplasm also is reviewed. Results: Excisional biopsy revealed primary ductal adenocarcinoma of the lacrimal gland. The patient subsequently underwent extensive local resection of the tumor and is awaiting radiotherapy to the orbit and neck. A literature search revealed only 3 prior reports of primary ductal adenocarcinoma of the lacrimal gland. Conclusions: Although uncommon, primary ductal adenocarcinoma of the lacrimal gland displays characteristic histologic and immunohistochemical findings that allow its distinction from the other primary lacrimal gland adenocarcinomas. Awareness and recognition of this rare malignancy may aid in further delineation of its biologic behavior, management, and prognosis. [Copyright &y& Elsevier]

Abstract: Sebaceous carcinoma of the ocular region is a malignant neoplasm that is being recognized more frequently and managed by innovative techniques of local resection, cryotherapy, topical chemotherapy, and radiotherapy, resulting in improved visual and systemic prognosis. [Copyright &y& Elsevier]

Objective: To describe clinical features, management, and prognosis of sebaceous carcinoma of the eyelid and adjacent structures. Design: Single-center retrospective interventional case series. Participants: Sixty consecutive patients with sebaceous carcinoma of the eyelid and adjacent structures. Methods: Retrospective chart review and literature review. Main outcome measures: Presenting features, sites of origin, location, growth patterns, management, histopathologic findings, incidence of recurrence, metastasis, and mortality. Results: The median age at referral was 72 years, with 73% female. Four patients had prior irradiation to the area where the sebaceous carcinoma developed. Initial clinical diagnoses elsewhere were sebaceous carcinoma (32%), blepharoconjunctivitis (25%), chalazion (20%), basal cell carcinoma (13%), and squamous cell carcinoma (10%). Initial histopathologic diagnoses elsewhere were sebaceous carcinoma (50%), squamous cell carcinoma (18%), basal cell carcinoma (8%), and others or not available (24%). Initial anatomic sites were upper eyelid (75%), lower eyelid (22%), caruncle (2%), and bulbar conjunctiva (2% [1 case]). Orbital exenteration was necessary in 13%. Recently introduced techniques of posterior lamellar resection of the eyelids with reconstruction (7%) hopefully will decrease this incidence in the future. Pathologically, 47% showed intraepithelial (pagetoid) involvement, 27% of sebaceous carcinomas arose from the meibomian glands, and 18% arose from both meibomian and Zeis glands. Local recurrence developed in 18%, metastasis in 8%, and death from metastasis in 6%. Conclusions: Despite the fact that the clinical features of sebaceous carcinoma have been widely reported, the diagnosis was suspected initially in only 32% of patients at first examination elsewhere and in only 50% at histopathologic examination elsewhere. Orbital exenteration was necessary in 13%, mostly patients seen in the earlier years of the study. With more recently employed treatment methods, there is a tendency to avoid exenteration and to use more conservative methods of treatment. It is hoped that these modern therapeutic approaches will result in fewer cases of recurrence and metastasis. [Copyright &y& Elsevier]

Purpose: To report clinical variations and the natural course of melanocytoma of the optic disc.Patients and methods: A retrospective study was conducted on a consecutive series of patients with melanocytoma of the optic disc who were evaluated at the Ocular Oncology Service at Wills Eye Hospital. Demographic information and variations in appearance and size of the tumor and local ocular complications were noted. Frequency of tumor growth and malignant transformation was recorded. Clinical factors, including patient data and tumor features, were analyzed for their impact on visual acuity loss of 2 or more Snellen lines and the tumor growth, using Cox proportional hazards regression models. Kaplan-Meier survival estimates of probability of visual acuity loss of 2 or more Snellen lines and probability of tumor growth were performed as a function of time from the initial examination.Main outcome measures: Visual acuity loss of 2 or more Snellen lines, tumor growth, and malignant transformation of the tumor.Results: There were 115 patients (116 eyes) with melanocytoma of the optic disc. The mean age at diagnosis was 50 years; 38% of patients were male and 62% of patients were female; 65% of patients were white, 29% of patients were African American, and 6% of patients were Asian, Hispanic, Indian, or Arabic. The lesion was unilateral in 99% of patients. Visual symptoms that seemed to be related to the melanocytoma were present in 24% of patients, and an afferent pupillary defect was noted in 9% of patients. Associated ocular abnormalities included ocular melanocytosis (8%), racial melanosis (7%), optic nerve hypoplasia (2%), and retinitis pigmentosa (1%). The melanocytoma was dark brown to black in 100% of patients. The mean tumor diameter was 2 mm and the mean thickness was 1 mm. Associated findings included a choroidal component (54%), retinal component (30%), optic disc edema (25%), retinal edema (16%), localized subretinal fluid (14%), retinal exudation (12%), retinal hemorrhage (5%), vitreous seeds (4%), and retinal vein obstruction (3%). Fluorescein and indocyanine green angiography typically showed persistent hypofluorescence of the lesion. Using Kaplan-Meier survival curves, related visual loss occurred in 18% of patients by 10 years, and minor tumor enlargement occurred in 11% of patients by 5 years and in 32% of patients by 10 years. Malignant transformation was documented in 2 patients (2%).Conclusions: Although melanocytoma of the optic disc generally is considered to be a benign, stationary lesion, it can produce several local complications, can cause visual loss, can grow slowly, and, rarely, can undergo malignant transformation into melanoma. Patients with optic disc melanocytoma should undergo periodic ocular examination. [Copyright &y& Elsevier]

: ObjectiveTo determine the incidence of specific orbital tumors based on patients referred to an ocular oncology center.: DesignRetrospective, observational case series.: ParticipantsA total of 1264 consecutive patients referred to an ocular oncology service because of space-occupying orbital lesions.: MethodsA retrospective chart review was carried out for 1264 consecutive patients referred for a suspected orbital mass over a 30-year period. The lesions were grouped into general categories, as shown in “Results.” The specific diagnosis in each case was based on clinical findings, computed tomography scan results, magnetic resonance imaging results, and histopathologic analysis results, when available. The number and percentage of benign and malignant tumors per age group also was determined.: Main outcome measuresThe incidence of orbital tumors and pseudotumors.: ResultsAmong 1264 consecutive patients, the number and percentage of lesions in each general category were as follows: cystic, 70 cases (6%); vasculogenic, 213 cases (17%); peripheral nerve lesions, 23 (2%); optic nerve and meningeal tumors, 105 (8%); fibrocytic lesions, 13 (1%); osseous and fibro-osseous tumors, 21 (2%); cartilaginous lesions, 1 (<1%); lipocytic and myxoid lesions, 64 (5%); myogenic tumors, 36 (3%); lacrimal gland lesions, 114 (9%); primary melanocytic lesions, 11 (<1%); metastatic tumors, 91 (7%); lymphoma and leukemia lesions, 130 (10%); secondary orbital tumors, 142 (11%); histiocytic lesions, 17 (1%); thyroid-related orbitopathy, 67 cases (5%); other inflammatory lesions, 133 cases (11%); and miscellaneous other lesions, 13 (1%). The most common diagnoses were: lymphoid tumor (139 cases;11%), idiopathic orbital inflammation (135 cases; 11%), cavernous hemangioma (77 cases; 6%), lymphangioma (54 cases; 4%), meningioma (53 cases; 4%), optic nerve glioma (48 cases; 4%), metastatic breast cancer (44 cases;4%), orbital extension of uveal melanoma (41 cases; 3%), capillary hemangioma (36 cases;3%), rhabdomyosarcoma (35 cases; 3%), dermolipoma (31 cases; 3%), herniated orbital fat (30 cases; 2%), dermoid cyst (26 cases; 2%), varix (26 cases; 2%), dacryops (19 cases; 2%), and other less common lesions. Of the 1264 lesions, 810 (64%) were benign and 454 (36%) were malignant. The percentage of malignant lesions was 20% in children (age range, 0–18 years), 27% in young adults and middle-aged patients (age range, 19–59 years), and 58% in older patents (age range, 60–92 years). Rhabdomyosarcoma was the most common malignancy in children, representing 3% of all orbital masses, and lymphoma was the most common malignancy in older patients, representing 10% of cases.: ConclusionsA variety of tumors and pseudotumors can involve the orbit. In this series of 1264 lesions, 64% were benign and 36% were malignant. The percentage of malignant tumors increased with age, with malignancies being common in older patients because of the higher incidence of lymphoma and metastasis in the elderly. [Copyright &y& Elsevier]

There is little in the literature about the clinical spectrum of orbital cysts of childhood and no comprehensive classification has been proposed. The authors propose a classification of orbital cysts of childhood and review their clinical features, pathology, and management. The major categories in the classification include cysts of surface epithelium, teratomatous cysts, neural cysts, secondary cysts, inflammatory cysts, and noncystic lesions with cystic component. Cysts of the surface epithelium are further divided into simple epithelial cyst (epidermal, conjunctival, respiratory, and apocrine gland), and dermoid cyst (epidermal and conjunctival). Epidermal dermoid cyst (dermoid) is by far the most common orbital cystic lesion in children, accounting for over 40% of all orbital lesions of childhood and for 89% of all orbital cystic lesions of childhood that come to biopsy or surgical removal. Neural cysts include those associated with ocular maldevelopment (congenital cystic eye and colobomatous cyst) and those associated with brain and meningeal tissue (cephalocele and optic nerve meningocele). The most important secondary cyst is mucocele that can occur in children with cystic fibrosis. Inflammatory cysts are generally due to parasitic infestations and are more common in tropical areas of the world. Noncystic lesions that can have a cystic component include adenoid cystic carcinoma, rhabdomyosarcoma, lymphangioma, and others. Each type of cyst has rather characteristic, but not pathognomonic, clinical features. Computed tomography and magnetic resonance imaging can help differentiate a cystic lesion from a solid tumor, suggest the type of cyst, and help in planning management. The pathology varies with the cells that line the cyst and with the inflammatory agent. Management varies from local excision to observation, depending on the location and type of cyst. Orbital cysts of childhood can be classified into categories, based mainly on their histopathology. The clinical, radiologic, and histopathologic features can be correlated with the classification in order to better evaluate a child with a cystic lesion in the orbit. [Copyright &y& Elsevier]

: PurposeTo document 2 cases of progressively enlarging retinal astrocytoma that caused exudative retinal detachment, vitreous hemorrhage, and tumor seeding, simulating retinoblastoma in 1 case and choroidal melanoma in the other.: DesignInterventional case reports.: ParticipantsTwo patients.: MethodsTwo patients with visual loss from an atypical, enlarging amelanotic retinal mass were evaluated. The first patient, a 14 year-old otherwise healthy girl, had a noncalcified gelatinous retinal mass with prepapillary involvement and surrounding retinal traction, as well as overlying vitreous hemorrhage and vitreous seeds. Over 37 months, the tumor grew in basal dimension from 4 mm to 10 mm. Retinal astrocytoma was suspected, but the presence of tumor enlargement and vitreous seeding raised concern for possible retinoblastoma. Fine-needle aspiration biopsy was performed. The second patient, a 33-year-old woman, had an amelanotic mass develop in the macula of her amblyopic left eye. Over 2 years, the mass enlarged to 9.5 mm in basal dimension and 6.3 mm in thickness, and total serous retinal detachment developed. Choroidal melanoma with retinal invasion was clinically suspected. Enucleation was performed.: ResultsIn patient 1, cytologic examination revealed bland spindle cells with fibrillar cytoplasm consistent with an astrocytic tumor of the retina. Enucleation was subsequently performed because of continued tumor growth, progressive retinal detachment, and visual loss. Histopathologic examination confirmed astrocytoma of the retina and optic disc. In patient 2, enucleation revealed astrocytoma of the retina and optic disc with total retinal detachment. There was no evidence of systemic tuberous sclerosis in either patient.: ConclusionsRetinal astrocytoma can show progressive enlargement, retinal detachment, and vitreous seeding, findings that can mislead the clinician toward the diagnosis of retinoblastoma or choroidal melanoma. [Copyright &y& Elsevier]

Tumors of the conjunctiva and cornea comprise a large and varied spectrum of conditions. These tumors are grouped into two major categories of congenital and acquired lesions. The acquired lesions are further subdivided based on origin of the mass into surface epithelial, melanocytic, vascular, fibrous, neural, histiocytic, myxoid, myogenic, lipomatous, lymphoid, leukemic, metastatic and secondary tumors. Melanocytic lesions include nevus, racial melanosis, primary acquired melanosis, melanoma, and other ocular surface conditions like ocular melanocytosis and secondary pigmentary deposition. The most frequent nonmelanocytic neoplastic lesions include squamous cell carcinoma and lymphoma, both of which have typical features appreciated on clinical examination. The caruncle displays a slightly different array of tumors compared to those elsewhere on the conjunctiva, as nevus and papilloma are most common, but oncocytoma and sebaceous gland hyperplasia, adenoma, and carcinoma can be found. In this report, we provide clinical description and illustration of the many conjunctival and corneal tumors and we discuss tumor management. [Copyright &y& Elsevier]

: PurposeTo report 5 patients with presumed congenital, simple hamartoma of the retinal pigment epithelium (RPE).: DesignRetrospective, observational small case series.: ParticipantsFive patients with a black macular tumor of the RPE.: Main outcome measuresInitial tumor features, initial associated fundus features, tumor course on follow-up, and visual acuity outcome.: ResultsIn all 5 cases, the tumor involved the macula and the mean distance to the foveola was 0.4 mm (median, 0.2 mm; range, 0.2–1.2 mm). In all cases, the tumor was darkly pigmented, with full-thickness retinal and RPE involvement and minimal protrusion into the vitreous cavity. The mean ultrasonographic tumor thickness was 1.6 mm (median, 1.4 mm; range, 1.1–2.5 mm). Associated features included minimally dilated retinal feeding artery and draining vein (100%), surrounding mild retinal traction (80%), retinal exudation (20%), and vitreous pigmented cells (20%). None of the lesions had associated subretinal fluid, macular edema, or macular hole. Visual acuity was 20/20 in 3 cases and slightly decreased in the other 2 cases because of foveal traction. The findings remained stable in 3 patients who had adequate follow-up. We chose to classify this lesion as congenital simple hamartoma of the RPE to differentiate it from combined hamartoma of the retina and RPE, congenital hypertrophy of the RPE, adenoma or adenocarcinoma of the RPE, and acquired hyperplasia of the RPE, which are different clinical entities.: ConclusionsCongenital hamartoma of the RPE is typically a black, full-thickness retinal mass, often adjacent to the foveola. Despite such location, the visual acuity was affected minimally. [Copyright &y& Elsevier]

Rhabdomyosarcoma is a malignant neoplasm that is composed of cells with histopathologic features of striated muscle in various stages of embryogenesis. It can occur in several sites in the body, including the ocular region. Ocular rhabdomyosarcoma is defined as the occurrence of this tumor in the area of the eye. Most ocular rhabdomyosarcomas arise in the soft tissues of the orbit but they can rarely occur in the other ocular adnexal structures and even within the eye. The purpose of this review is to provide a brief overview of rhabdomyosarcoma and a more detailed review of orbital rhabdomyosarcoma, with emphasis on changing concepts in the diagnosis and management of this ophthalmic neoplasm. [Copyright &y& Elsevier]

: ObjectiveThe objective of this report was to evaluate ocular and systemic outcomes after primary transpupillary thermotherapy for choroidal melanoma and to identify the limitations of this treatment method.: DesignNoncomparative interventional case series.: ParticipantsThe participants included 256 patients with newly diagnosed choroidal melanoma.: Main outcome measuresThe outcome measures included local tumor recurrence and visual acuity.: ResultsBefore treatment, the mean tumor base was 7.1 mm, and mean tumor thickness was 2.7 mm. Overlying subretinal fluid was present in 215 cases (84%) and orange pigment on the tumor surface in 200 cases (78%). All tumors showed either photographic documentation of growth (114 cases; 45%) or substantial risk factors for growth (142 cases; 55%). After a mean of three treatment sessions, complete tumor control without recurrence was found in 232 cases (91%) and recurrence in 24 cases (9%). The mean time to recurrence was 22 months, and the mean recurrent tumor size was 3.8 mm base and 2.4 mm thick. Of the recurrent tumors, additional thermotherapy was successful in controlling 13 (5%), plaque radiotherapy in 8 (3%), and enucleation in 3 (1%). Using multivariable analysis, the risk factors for tumor recurrence included increasing number of thermotherapy sessions (reflecting less responsive tumor) (P = 0.0001) and optic disc overhung by tumor (P = 0.03). Kaplan Meier estimates revealed that 4% showed recurrence at 1 year, 12% at 2 years, and 22% at 3 years follow-up. When analyzing those 214 patients without multivariable risk factors for recurrence, Kaplan Meier estimates for recurrence were 2% at 1 year, 8% at 2 years, and 10% at 3 years. The visual acuity after treatment was 20/20 to 20/40 in 128 cases (50%), 20/50 to 20/100 in 47 (18%), and 20/200 or worse in 81 (32%). Using multivariable analysis, the most statistically significant factors at initial visit that were predictive of poor visual acuity (20/200 or worse) after treatment included documented tumor growth before treatment (P = 0.0001), mushroom tumor configuration (P = 0.002), initial symptom of blurred vision (P = 0.008), poor initial visual acuity (P = 0.005), superior quadrant tumor location (P = 0.03), underlying diabetes mellitus (P = 0.04), and optic disc overhung by tumor (P = 0.04). Tumor-related mortality occurred in two patients (1%), one of whom showed complete tumor regression to thermotherapy and the other with diffuse choroidal melanoma and local tumor margin recurrence.: ConclusionsTranspupillary thermotherapy is an effective treatment for certain small choroidal melanomas. Appropriate tumor selection is critical to successful treatment. Patients with tumors abutting or overhanging the optic disc or those requiring more than three sessions for tumor control are more likely to develop ultimate tumor recurrence. Transpupillary thermotherapy can cause damaging effects to the retina, leading to visual loss shortly after treatment. [Copyright &y& Elsevier]

There are many lesions that can present as an elevation of the optic disc. Since each of them has different ophthalmic and systemic significance, it is important for the ophthalmologist to recognize them and to plan strategies of management when necessary. Important inflammatory and benign congenital conditions should be differentiated from malignant neoplasms. This review covers the differential diagnosis of lesions that can cause an elevation of the optic disc. It includes selected congenital lesions, inflammatory conditions, non-inflammatory non-neoplastic disorders, benign hamartomas, and primary and secondary malignant neoplasms. [ABSTRACT FROM AUTHOR]

Background Methods Results Conclusion Uveal melanoma (UM) can be classified by tumour size category and by The Cancer Genome Atlas (TCGA) groups (cytogenetic‐based, 4‐category prognostic classification into Groups A‐D). This study was conducted to assess impact on metastasis‐free survival (MFS) in UM by tumour size category based on correlation with TCGA classification.Retrospective analysis of 1001 cases categorised as small (0.0–3.0 mm), medium (3.1–8.0 mm) and large (≥8.1 mm), grouped by TCGA classification.Of 1001 cases, TCGA Groups (A/B/C/D) included small (n = 270, 75%/11%/13%/1%), medium (n = 503, 46%/14%/27%/13%) and large (n = 228, 23%/19%/38%/20%) UM. The 5‐and 10‐year Kaplan–Meier MFS for small UM revealed Group A (98%, 98%), Group B (100%, 100%), Group C (86%, NA) and Group D (100%, NA). For medium UM, the values dropped with Group A (95%, 93%), Group B (90%, 90%), Group C (68%, 38%), and Group D (44%, NA). For large UM, the values dropped further with Group A (94%, 86%), Group B (85%, NA), Group C (40%, 28%), and Group D (23%, NA). Additionally, a comparison (small vs. medium vs. large tumour size category) revealed TCGA low‐risk grouping (Groups A or B) in 86% vs. 60% vs. 58% cases with UM.By tumour size category, favourable cytogenetics (Groups A or B) is found in 86% of small tumours, 60% of medium tumours, and 58% of large tumours. The MFS at 10 years for favourable cytogenetics was 98% for small tumours, 92% for medium tumours, and 54% for large tumours. Tumour size category can serve as a surrogate for TCGA. [ABSTRACT FROM AUTHOR]

Biopsy elsewhere revealed eyelid squamous cell carcinoma (SCC), well-differentiated. These findings were compatible with mucoepidermoid carcinoma, now referred to as adenosquamous carcinoma by the World Health Organization.[[1]] Conjunctival adenosquamous carcinoma is rare, accounting for <1% of all conjunctival tumors.[[2]] This malignancy develops in the conjunctiva as a gray-white mass, typically in older patients and with an aggressive course. [Extracted from the article]

Intraocular medulloepithelioma is a nonhereditary neoplasm of childhood arising from primitive medullary epithelium. It most often involves the ciliary body. Most patients present between 2 and 10 years of age with loss of vision, pain, leucocoria, or conjunctival congestion. The mass appears as a grey-white ciliary body lesion with intratumoral cysts. Presence of a neoplastic cyclitic membrane with extension to retrolental region is characteristic. Secondary manifestations like cataract and neovascular glaucoma may be present in up to 50% and 60% patients, respectively. These could be the first signs for which, unfortunately, about 50% patients undergo surgery before recognition of the hidden tumor. Systemic correlation with pleuropulmonary blastoma (DICER1 gene) has been documented in 5% cases. Histopathology shows primitive neuroepithelial cells arranged as cords closely resembling the primitive retina. Histopathologically, the tumor is classified as teratoid (containing heteroplastic elements) and nonteratoid (containing medullary epithelial elements), each of which are further subclassified as benign or malignant. Retinoblastoma-like and sarcoma-like areas may be seen within the tissue. The treatment modality depends on tumor size and extent of invasion. For small localized tumors (≤3-4 clock hours), conservative treatments with cryotherapy, plaque radiotherapy, or partial lamellar sclerouvectomy (PLSU) have been used. Plaque brachytherapy is generally preferred for best tumor control. Advanced and extensive tumors require enucleation. Rare use of intra-arterial and intravitreal chemotherapy has been employed. Systemic prognosis is favorable, but those with extraocular extension and orbital involvement show risk for local recurrence and metastatic disease, which can lead to death. [ABSTRACT FROM AUTHOR]

An introduction to the article is presented in which editor discusses various issues within the article including removal of melanoma tumors surgically, resection of choroidal neoplasms and dry removal of tumor by fine cautery.

A letter to the editor is presented about retinal vasoproliferative tumor as a manifestation of retinitis pigmentosa.

Purpose: To report two glomangiomas in one orbit, each isolated to a rectus muscle. Design: Clinicopathologic correlation. Methods: A 12-year-old boy developed two separate vascular tumors, near the insertions of the medial rectus and superior rectus muscles, respectively. A biopsy of one tumor was studied by light microscopy and immunohistochemistry. Results: Histopathology revealed blood vessels surrounded by cuboidal cells characteristic of glomangioma. The cells showed immunoreactivity for smooth muscle actin and vimentin, supporting the diagnosis. Conclusions: Glomangioma can involve the rectus muscles in the conjunctiva and orbit, and should be considered in differential diagnosis of vascular tumors in the ocular region. [Copyright &y& Elsevier]

Purpose: To report choroidal metastasis from medullary thyroid carcinoma in a patient with multiple endocrine neoplasia (MEN) type 2b.Design: Interventional case report.Methods: Medullary thyroid carcinoma developed in a 20-year-old woman and led to the diagnosis of MEN 2b. She had mucosal neuromas on the lips and tongue and prominent corneal nerves in both eyes. Right choroidal metastasis developed when she was 36 years of age.Results: The eye was treated with irradiation, but 9 months later the patient died of widespread metastases.Conclusions: In patients with MEN type 2b, choroidal metastasis can develop from medullary thyroid carcinoma. Although fundus abnormalities typically do not occur with MEN 2b, choroidal metastasis from medullary thyroid carcinoma is a potential fundus finding. [ABSTRACT FROM AUTHOR]

Purpose: To document progressive growth of a benign iris melanocytoma.Methods: Interventional case report. A 9-year-old male underwent removal of a pigmented iris tumor that had been documented photographically to double in size.Results: Histopathologic sections revealed a deeply pigmented mass with cytologic features typical of a melanocytoma.Conclusion: Although iris melanocytoma is generally a stationary lesion, it can show progressive growth. Such enlargement does not necessarily imply malignant transformation of the lesion. [ABSTRACT FROM AUTHOR]

Purpose: To understand the prognostic value of The Cancer Genome Atlas (TCGA) for uveal melanoma metastasis, using a simplified 4-category classification, based on tumor DNA.Methods: A retrospective cohort study of 1001 eyes with uveal melanoma at a single center, categorized according to TCGA as Group A, B, C, or D (by fine-needle aspiration biopsy for DNA analysis), and treated with standard methods, was studied for melanoma-related metastasis at 5 and 10 years.Results: Of 1001 eyes with uveal melanoma, the TCGA categories included Group A (n = 486, 49%), B (n = 141, 14%), C (n = 260, 26%), and D (n = 114, 11%). By comparison, increasing category (A vs. B vs. C vs. D) was associated with features of older age at presentation (56.8 vs. 52.8 vs. 61.1 vs. 63.5 years, P < 0.001), less often visual acuity of 20/20-20/50 (80% vs. 67% vs. 70% vs. 65%, P = 0.001), tumor location further from the optic disc (P < 0.001) and foveola (P < 0.001), and greater median tumor basal diameter (10.0 vs. 13.0 vs. 14.0 vs. 16.0 mm, P < 0.001) and tumor thickness (3.5 vs. 5.2 vs. 6.0 vs. 7.1 mm, P < 0.001). The Kaplan-Meier (5-year/10-year) rate of metastasis was 4%/6% for Group A, 12%/20% for Group B, 33%/49% for Group C, and 60%/not available for Group D.Conclusion: A simplified 4-category classification of uveal melanoma using TCGA, based on tumor DNA, is highly predictive of risk for metastatic disease. [ABSTRACT FROM AUTHOR]

Purpose: The aim of this study was to evaluate tumor control of OSSN with topical IFNα2b alone based on tumor configuration (flat versus (vs.) dome-shaped).Methods: Retrospective, nonrandomized, interventional cohort study on 64 consecutive tumors in 63 patients with OSSN treated with topical IFNα2b. Topical IFNα2b (1 million international units/cc) was compounded and provided by the Thomas Jefferson University Hospital Pharmacy to be refrigerated and applied 4 times daily until biomicroscopic evidence of tumor resolution was observed.Results: The tumor configuration was flat (n = 15, 23%) or dome-shaped (n = 49, 77%). A comparison (flat vs. dome-shaped) revealed dome-shaped with older mean patient age at presentation (62 vs. 70 years, P = 0.04), greater patient history of smoking (13% vs. 42%, P = 0.04), greater corneal involvement (7% vs. 82%, P < 0.001), larger mean basal diameter (5.5 vs. 12.4 mm, P = 0.001) and mean thickness (1.9 vs. 4.3, P = 0.002), and longer mean duration IFNα2b therapy (3.7 vs. 6.3 months, P = 0.002). There was no difference in mean follow-up time (22.2 vs 23.1 months) or time to complete response (5.0 vs. 6.1 months). There was no difference in achievement of complete tumor control with IFNα2b alone (93% vs. 96%). There were no cases with metastasis or death.Conclusion: Topical IFNα2b alone shows excellent overall tumor control of 95% with no difference in efficacy based on tumor configuration. [ABSTRACT FROM AUTHOR]

Purpose: To evaluate choroidal nevus demographics, clinical features, imaging features, and the rate of transformation into melanoma by race.Methods: In this observational case series,: There were 3334 participants (3806 choroidal nevi) at a single tertiary-referral center evaluated between January 2, 2007, and August 7, 2017. Retrospective chart and multimodal imaging review was performed. Patient demographics, tumor features, and outcomes were compared between different races using Chi-squared test, Fisher's exact test, t-test, and analysis of variance. The main outcome measure was clinical features of choroidal nevus and the rate of transformation into melanoma by race.Results: Of the 3334 patients, there were Caucasian (n = 3167, 95%) and non-Caucasian (n = 167, 5%). The non-Caucasian races included African-American (n = 27, <1%), Hispanic (n = 38, <1%), Asian (n = 15, <1%), Asian Indian (n = 2, <1%), Middle Eastern (n = 4, <1%), and unknown (n = 83, 3%). By comparison (Caucasian versus vs. non-Caucasian), there were differences in the mean age at presentation (61 vs. 56 years,P < 0.0001), female sex (63% vs. 52%,P < 0.01), dysplastic nevus syndrome (<1% vs. 1%,P < 0.01), and previous cutaneous melanoma (5% vs. 1%,P= 0.03). A comparison of tumor features revealed differences in presence of symptoms (12% vs. 20%,P < 0.01) and ≥3 nevi per eye (3% vs. <1%,P= 0.04). A comparison of imaging features showed no differences. A comparison of outcome of nevus transformation into melanoma revealed no difference (2% vs. 3%,P= 0.29). However, of those nevi exhibiting growth to melanoma, ultrasonographic hollowness was less frequent in Caucasians (29% vs. 67%,P= 0.04).Conclusion: In this analysis of 3334 patients with choroidal nevus, we found differences in the mean age of presentation, sex, dysplastic nevus syndrome, previous cutaneous melanoma, presence of symptoms, and multiplicity of nevus per eye by race. However, there was no difference in the rate of transformation into melanoma by race. [ABSTRACT FROM AUTHOR]

Purpose: The cancer genome atlas (TCGA) is a comprehensive project supported by the National Cancer Institute (NCI) in the United States to explore molecular alterations in cancer, including uveal melanoma (UM). This led to TCGA classification for UM. In this report, we review the American Joint Committee on Cancer (AJCC) classification and TCGA classification for UM from the NCI's Center for Cancer Genomics (NCI CCG) (based on enucleation specimens [n = 80 eyes]) and from Wills Eye Hospital (WEH) (based on fine needle aspiration biopsy [FNAB] specimens [n = 658 eyes]). We then compare accuracy and predictability of AJCC versus (vs.) TCGA.Methods: Review of published reports on AJCC and TCGA classification for UM was performed. Outcomes based on AJCC 7th and 8th editions were assessed. For TCGA, UM was classified based on chromosomes 3 and 8 findings including disomy 3 (D3), monosomy 3 (M3), disomy 8 (D8), 8q gain (8qG), or 8q gain multiple (8qGm) and combined into four classes including Class A (D3/D8), Class B (D3/8qG), Class C (M3/8qG), and Class D (M3/8qGm). Outcomes of metastasis and death were explored and a comparison (AJCC vs. TCGA) was performed.Results: In the NCI CCG study, there were 80 eyes with UM sampled by enucleation (n = 77), resection (n = 2), or orbitotomy (n = 1) and analysis revealed four distinct genetic classes. Metastasis and death outcomes were subsequently evaluated per class in the WEH study. The WEH study reviewed 658 eyes with UM, sampled by FNAB, and found Class A (n = 342, 52%), B (n = 91, 14%), C (n = 118, 18%), and D (n = 107, 16%). Comparison by increasing class (A vs. B vs. C vs. D) revealed older mean patient age (P < 0.001), worse entering visual acuity (P < 0.001), greater distance from the optic disc (P < 0.001), larger tumor diameter (P < 0.001), and greater tumor thickness (P < 0.001). Regarding outcomes, more advanced TCGA class demonstrated increased 5-year risk for metastasis (4% vs. 20% vs. 33% vs. 63%,P < 0.001) with corresponding increasing hazard ratio (HR) (1.0 vs. 4.1, 10.1, 30.0,P= 0.01 for B vs. A andP < 0.001 for C vs. A and D vs. A) as well as increased 5-year estimated risk for death (1% vs. 0% vs. 9% vs. 23%,P < 0.001) with corresponding increasing HR (1 vs. NA vs. 3.1 vs. 13.7,P= 0.11 for C vs. A andP < 0.001 for D vs. A). Comparison of AJCC to TCGA classification revealed TCGA was superior in prediction of metastasis and death from UM.Conclusion: TCGA classification for UM is simple, accurate, and highly predictive of melanoma-related metastasis and death, more so than the AJCC classification. [ABSTRACT FROM AUTHOR]

A choroidal nevus is a common intraocular tumor in the United States, found in approximately 5% of Caucasian adults. The three main risks of melanocytic choroidal nevus include vision loss from a subfoveal nevus, development of subretinal fluid, and transformation of nevus into melanoma, a malignant counterpart. We explore clinical risk factors that predict benign melanocytic choroidal nevus transformation into a malignant choroidal melanoma. Based on a large analysis of 2,355 cases that were monitored longitudinally using multimodal imaging, the most recent list of clinical features includes tumor Thickness greater than 2 mm on ultrasonography, subretinal Fluid on optical coherence tomography, Symptomatic vision loss 20/50 or worse, Orange pigment on fundus autofluorescence, Melanoma hollow on ultrasonography, and DIaMeter greater than 5 mm on fundus photography. These factors are remembered with a mnemonic of the capital letters TFSOM-DIM for "To Find Small Ocular Melanoma Doing Imaging." Analysis of these factors demonstrated a Kaplan-Meier mean five-year risk of 1% with no risk factors, 11% with any one factor, 22% with any two factors, 34% with any three factors, 51% with any four factors, and 55% with any five factors. There was no patient with six risk factors. Of those with combinations of four risk factors, six of 15 combinations yielded a 70%-100% rate of transformation; of those with combinations of five risk factors, two of five combinations yielded a 70%-100% rate of transformation. Choroidal nevus carries a risk for evolving into melanoma, and understanding of clinical and imaging features predictive of this outcome is highly important. [ABSTRACT FROM AUTHOR]

Conjunctival melanoma is quite rare, estimated at approximately 0.5 incidence per 1 million persons per year. This malignancy arises from a pre-existing nevus (7%), primary acquired melanosis (74%), or de novo without pre-existing condition (19%) and develops most often in patients with Fitzpatrick skin types I (23%) and II (62%). At initial presentation, the tumor size is approximately 13 mm in cross-sectional diameter and has 3-mm thickness, involving the bulbar (97%), forniceal (30%), tarsal (28%), or caruncular (11%) regions, often with corneal (54%) and rarely with orbital (4%) involvement. According to the eighth edition of the American Joint Committee on Cancer (AJCC), the tumor is classified as T1 (63%), T2 (18%), T3 (20%), and T4 (0%). Outcomes depend on several factors including patient age, AJCC classification, orbital invasion, and type of initial surgery, whereas tumor origin and Fitzpatrick skin type do not appear to impact outcomes. Older patients (≥70 years of age) demonstrate larger tumors, greater recurrence, and greater vision loss. Analysis of 425 patients by AJCC classification (T1 versus T2 versus T3) revealed increasing T category with greater lymph node metastasis (3% versus 13% versus 25%; P <.001), tumor-related systemic metastasis (13% versus 45% versus 40%; P <.001), and tumor-related death (8% versus 22% versus 37%; P <.001). Data of patients with orbital invasion revealed significantly greater 10-year rates of exenteration (P <.001), distant metastasis (P =.0005), and death (P =.001). Studies have demonstrated biomarkers related to conjunctival melanoma include mutations in BRAF, NRAS, ATRX, and NF1. Future therapies might be directed against these mutations or with small-molecule inhibitors and/or immunotherapy. In summary, conjunctival melanoma is a rare but ominous malignancy, imparting moderate risk for lymph node and systemic metastasis as well as death, depending on tumor features and classification. The first surgery is highly important in prevention of tumor seeding, recurrence, and metastasis. [ABSTRACT FROM AUTHOR]

A letter to the editor and its response to the article "Reactive Retinal Astrocytic Tumors (So-called Vasoproliferative Tumors): Histopathologic, Immunohistochemical, and Genetic Studies of Four Cases'' by Poole Perry and associates in the July, 2013 issue is presented.

Purpose: To assess features and outcomes of Coats disease over 5-decades.Methods: Retrospective review of Coats disease patients at a single center. Features and outcomes were compared based on decade of presentation.Results: There were 351 patients with Coats disease. The presenting median age (6 years), male sex (84%), and unilaterality (100%) did not change per decade. Coats disease classification did not change per decade with Stage 1 (1%), Stage 2 (21%), Stage 3 (68%), Stage 4 (6%), and Stage 5 (1%). Clinical features that changed per decade (1970s vs. 1980s vs. 1990s vs. 2000s vs. 2010s) included 1980s features of more eyes with exudation in all 4 quadrants (22% vs. 58% vs. 44% vs. 33% vs. 27, P = 0.01) and total exudative retinal detachment (33% vs. 53% vs. 39% vs. 27% vs. 21%, P < 0.001). Imaging features that changed per decade included 2010s greater fluorescein angiographic extent of retinal non-perfusion in mean clock hours (4 vs. 4 vs. 3 vs. 5 vs. 6, P = 0.003), and 1980s greater mean height of retinal detachment ultrasonographically (5 vs. 12 vs. 5 vs. 5 vs. 4 mm, P < 0.001). Treatment features that changed per decade included 1980s greater primary enucleation (11% vs. 16% vs. 3% vs. 4% vs. 1%, P = 0.001), and 2010s greater use of laser photocoagulation (55% vs. 33% vs. 38% vs. 40% vs. 72%, P < 0.001), sub-Tenon corticosteroid (0% vs. 4% vs. 5% vs. 8% vs. 29%, P < 0.001), and intravitreal anti-VEGF) (0% vs. 4% vs. 2% vs. 13% vs. 18%, P = 0.003). Outcomes that changed per decade included 2010s findings of more complete resolution of subretinal fluid (64% vs. 59% vs. 38% vs. 58% vs. 72%, P = 0.01) and less need for primary/secondary enucleation (17% vs. 27% vs. 14% vs. 13% vs. 6%, P = 0.04).Conclusion: Eyes with Coats disease in the 1980s demonstrated more advanced findings, often requiring enucleation. Over the decades, greater use of laser photocoagulation and injections has led to improved disease resolution with greater globe salvage. [ABSTRACT FROM AUTHOR]

Coats disease is an idiopathic retinal vascular disorder with retinal telangiectasia with intraretinal and/or subretinal exudation without appreciable retinal or vitreal traction. The condition is sporadic with no associated systemic abnormalities. Unilateral involvement in young males is the typical presentation with most cases being diagnosed in the first and second decade of life. Younger the patient, more severe is the presentation and poorer the visual outcome. The management varies with the stage of the disease. Over the years, we have shifted from enucleation to a more conservative approach for the treatment of Coats disease with laser photocoagulation, cryotherapy and surgery for retinal detachment achieving good outcomes. The anti-VEGF agents have come into the scene as important form of adjuvant treatment along with the traditional management options. This article describes the clinical features, underlying pathology, classification and staging, the complications and the management of Coats disease and gives an overview of the changing trends in treatment and outcomes spanning across five decades. [ABSTRACT FROM AUTHOR]

Presents the results of a clinical study about the characteristics of patients developing Coats' Disease in the U.S. Number of patients who participated in the study; Percentage of patients with localized hemorrhage; Information on a problem in the diagnosis of Coats' disease in adults.

Purpose: To describe total blindness secondary to presumed optic nerve melanocytoma. Design: Interventional case report. Methods: A 12-year-old African-American girl, who developed loss of light perception secondary to a pigmented lesion of the optic disk, underwent transvitreal fine-needle aspiration biopsy (FNAB) of the lesion. Results: Cytopathology revealed cells with features of melanocytoma and melanophages but no cells compatible with melanoma. The patient is being followed without treatment. Conclusions: Optic nerve melanocytoma can cause total blindness in the affected eye. Although it has limitations, FNAB can assist in diagnosis and management of melanocytoma with visual loss. [Copyright &y& Elsevier]