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Conjunctival tumors include a spectrum of benign and malignant neoplasms. (1-5) The types differ based on age and race, systemic immune status and long-term exposures. A large study of 5,002 [...]

The greatest likelihood of a germline mutation in patients with a unilateral retinoblastoma occurred in patients who were 1 year or younger versus those older than 1 year, as well [...]

A practical approach to management of retinoblastoma is presented using the basic clinical features. This approach is predictive of treatment success for eyes in which modern conservative therapy for retinoblastoma is used.

Objective: To identify the clinical features of eyes with retinoblastomas that predict the recurrence of retinal tumors, vitreous seeds, and subretinal seeds following treatment with chemoreduction. Design: Prospective nonrandomized single-center clinical trial. Setting: Ocular oncology service at Wills Eye Hospital of Thomas Jefferson University (Philadelphia, Pa) in conjunction with the division of oncology at Children's Hospital of Philadelphia. Participants: There were 158 eyes with 364 tumors in 103 consecutive patients with retinoblastoma managed with chemoreduction between June 1994 and August 1999. Intervention: All patients received treatment for retinoblastoma with 6 cycles of chemoreduction using vincristine, etoposide, and carboplatin combined with focal treatment (cryotherapy, thermotherapy, or plaque radiotherapy) for each retinal tumor. Main Outcome Measures: The 3 main outcome measures included recurrence of retinal tumors, recurrence of vitreous seeds, and recurrence of subretinal seeds. The clinical features at the initial examination were analyzed for their association with the main outcome measures using a series of Cox proportional hazards regressions. Results: All retinal tumors, vitreous seeds, and subretinal seeds showed an initial favorable response of regression during this treatment regimen. Using Kaplan-Meier estimates, at least 1 retinal tumor recurrence per eye was found in 37% of eyes at i year, 51% at 3 years, and no further increase at 5 years. By multivariate analysis, the only factor predictive of retinal tumor recurrence was the presence of tumor-associated subretinal seeds at the initial examination. Of the 54 eyes that had vitreous seeds at the initial examination, vitreous seed recurrence was found in 26% of eyes at 1 year, 46% at 3 years, and 50% at 5 years. By univariate analysis, the only factor predictive of vitreous seed recurrence was the presence of tumor-associated subretinal seeds at the initial examination. Of the 71 eyes that had subretinal seeds at the initial examination, subretinal seed recurrence was detected in 53% of eyes at 1 year, 62% at 3 years, and no further increase at 5 years. By multivariate analysis, factors predictive of subretinal seed recurrence included a tumor base greater than 15 mm and a patient age of 12 months or younger at diagnosis. There were no patients who developed retinoblastoma metastasis, pinealoblastoma, or second malignant neoplasms. Conclusions: Chemoreduction combined with focal therapy is effective for selected eyes with retinoblastomas. Eyes with subretinal seeds at initial examination are at particular risk for recurrence of retinal tumor and vitreous seeds. Younger patients with large tumors are at risk for recurrence of subretinal seeds. Retinal tumor and subretinal seed recurrence seems to manifest within 3 years of follow-up. Close follow-up of all patients treated with chemoreduction is warranted. Arch Ophthalmol. 2002;120:460-464

Objective: To report the clinical characteristics of solitary idiopathic choroiditis and the features that differentiate it from tumors and other inflammatory lesions. Design: Retrospective medical record review. Patients: Sixty consecutive patients with solitary idiopathic choroiditis. Main Outcome Measures: Clinical features, natural course, and follow-up. Results: The mean and median ages of the patients were 35 and 36 years, respectively, 56 (93%) of the 60 patients were white, 38 (63%) were female, and 22 (37%) were male. No patient had a history, clinical findings, or laboratory study results to support a specific cause of uveitis. All were referred to us because of a suspected intraocular tumor. The patient was asymptomatic in 21 cases (35%); the patient had mild visual loss in 36 cases (60%). The lesion was posterior to the equator in 56 cases (93%), was yellow in 58 cases (97%), and had distinct margins in 38 cases (63%). The lesion showed signs of active inflammation in 20 cases (33%) and no inflammation in 40 cases (67%). Fluorescein angiography disclosed that all lesions had early hypofluorescence and late hyperfluorescence. In the late-phase angiograms inactive lesions had distinct margins and active lesions had ill-defined margins. Lesions with active inflammation appeared to show a favorable response to the administration of systemic corticosteroids but generally improved with or without treatment. Most of the inactive lesions remained stable on long-term follow-up. Overall, the condition remained stable in 36 patients (60%), improved in 22 (37%), and recurred in 2 (3%). The clinical and angiographic features and clinical course of solitary idiopathic choroiditis were generally different from known intraocular tumors. Conclusions: Solitary idiopathic choroiditis is a distinct clinical entity that can simulate an intraocular neoplasm. Recognition of its typical clinical features can assist in differentiating it from tumors and other inflammatory lesions of the ocular fundus. Arch Ophthalmol. 2002; 120:311-319

Objectives: To analyze the results of intraocular surgery in patients treated for retinoblastoma and to assess the ocular and systemic outcomes. Design: Retrospective noncomparative case series. Patients: Forty-five consecutive patients who underwent an introcular surgery after treatment for retinoblastoma. Main Outcome Measures: (1) Recurrence of retinoblastoma, (2) need for enucleation, and (3) systemic metastasis. Overall outcome was defined as favorable in the absence of any of these measures and unfavorable in the presence of 1 or more. Results: Thirty-four patients (76%) underwent a single procedure of cataract surgery, a scleral buckling procedure, or pars plana vitrectomy and 11 (24%) underwent a combination of 2 or more surgical procedures. In all, 16 patients (36%) achieved final visual acuity better than 20/200. Unfavorable outcomes included recurrence of retinoblastoma in 14 patients (31%), enucleation in 16 (36%), and systemic metastasis in 3 (7%). Five patients (20%) who underwent cataract surgery, 5 (63%) who underwent a scleral buckling procedure, and 9 (75%) who underwent pars plana vitrectomy manifested an unfavorable outcome. The median interval between completion of treatment for retinoblastoma and intraocular surgery was 26 months in patients with a favorable outcome vs 6 months in those with an unfavorable outcome. Conclusions: Intraocular surgery after treatment for retinoblastoma may be justified in certain exceptional clinical situations. Cataract surgery is safe and effective in most cases. However, the need for a scleral buckling procedure and pars plana vitrectomy may be associated with a higher risk for recurrence of retinoblastoma, enucleation, and systemic metastasis, and a cautious approach is warranted. Arch Ophthalmol. 2001;119:1613-1621

Background. Optic nerve invasion is one of the predictors for retinoblastoma metastases. This study was designed to investigate the risk of optic nerve invasion and clinical features that may identify those children with optic nerve invasion. Methods. We reviewed the charts of 289 children with retinoblastoma treated initially with enucleation. Logistic regression analysis was performed to assess the risk for metastases from varying degrees of optic nerve invasion and to assess the clinical and histopathologic predictors of optic nerve invasion. Results. There were 84 eyes (29%) with optic nerve invasion. The invasion was prelamina cribrosa in 44 cases (15%), up to but not posterior to the lamina cribrosa in 21 cases (7%), posterior to the lamina cribrosa but not to the cut end of the optic nerve in 17 cases (6%), and to the site of optic nerve transection in 2 cases (1%). Patients with optic nerve invasion were more likely to develop metastasis (P = 0.0016), particularly those with invasion to the postlaminar and cut section of the optic nerve (P = 0.0001). Development of metastasis was not statistically associated with laminar or prelaminar involvement. If those patients with choroidal invasion simultaneous with optic nerve invasion were excluded from evaluation, the presence of optic nerve invasion alone was not significant for development of metastasis. The clinical factors found to be predictive for optic nerve invasion from a univariate analysis included exophytic growth pattern (P = 0.011), elevated intraocular pressure (> 22 mm Hg) (P = 0.02), and tumor thickness greater than or equal to 15 mm (P = 0.03). The histopathologic factor significantly associated with optic nerve invasion (univariate analysis) was simultaneous choroidal invasion (P = 0.001). A trend toward an association with optic nerve invasion was found with vitreous hemorrhage (P = 0.06), iris neovascularization (P = 0.10), and poorly differentiated retinoblastoma (P = 0.07). A multivariate analysis showed the most significant clinical factors to be exophytic growth pattern (P = 0.002), tumor thickness greater than or equal to 15 mm (P = 0.01), and vitreous hemorrhage (P = 0.05). Conclusions. Optic nerve invasion of retinoblastoma beyond the lamina cribrosa is associated with a greater metastatic risk. Large exophytic retinoblastoma with secondary glaucoma is at highest risk for optic nerve invasion.

Background. Although breast and lung cancers account for most metastatic tumors to the uvea, prostate carcinoma metastatic to the uvea is relatively rare. Methods. The authors reviewed 379 consecutive patients with uveal metastasis referred to the Ocular Oncology Service at the Wills Eye Hospital to determine the frequency of prostate carcinoma as the primary neoplasm. Results. Of 379 consecutive patients with uveal metastasis, the primary tumor was prostate carcinoma in 7 (2% of all patients, 5% of male patients). The mean age at ocular diagnosis was 66 years. In six patients (85%) a prior history of prostate carcinoma was known and the mean time interval between the diagnosis of the primary tumor and the uveal metastasis was 28 months. All seven patients were found to have active or regressed metastases elsewhere. The choroid was involved in six patients (85%) and the iris in one (15%). Ocular treatment included external-beam radiation therapy in five patients (70%) and episcleral radioactive plaque in two (30%). After a total mean follow-up of 84 months (range, 44-140 months) from the diagnosis of prostate carcinoma, two patients were still alive and five patients have died (mean, 6 months after diagnosis of uveal metastasis). Conclusions. Prostate carcinoma can metastasize to the uvea and the presence of uveal metastasis is associated with a shorter survival time compared with metastasis to other sites. Cancer 1993; 71:2791-6.