Intravitreal bevacizumab at 4-month intervals for prevention of macular edema after plaque radiotherapy of uveal melanoma.

PURPOSE: To evaluate the efficacy of intravitreal bevacizumab for prevention of macular edema after plaque radiotherapy of uveal melanoma. DESIGN: Retrospective, single-center, nonrandomized, interventional comparative study. PARTICIPANTS: Patients with uveal melanoma treated with plaque radiotherapy were divided into 2 groups: a bevacizumab group and a control group. INTERVENTION: The bevacizumab group received intravitreal bevacizumab injection at the time of plaque removal and every 4 months thereafter for 2 years (total, 7 injections). The control group had no intravitreal bevacizumab injection. Both groups had periodic follow-up with ophthalmoscopy and optical coherence tomography (OCT). MAIN OUTCOME MEASURES: Development of OCT-evident macular edema. RESULTS: There were 292 patients in the bevacizumab group and 126 in the control group. The median foveolar radiation dose was 4292 cGy (bevacizumab) and 4038 cGy (control; P = 0.327). The cumulative incidence of OCT-evident macular edema over 2 years (bevacizumab group vs. control group) was 26% versus 40% (P = 0.004), respectively; that for clinically evident radiation maculopathy was 16% versus 31% (P = 0.001), respectively; that for moderate vision loss was 33% versus 57% (P < 0.001), respectively; and that for poor visual acuity was 15% versus 28% (P = 0.004), respectively. There was no statistically significant difference in clinically evident radiation papillopathy (P = 0.422). Kaplan-Meier estimates at 2 years showed statistically significantly reduced rates of OCT-evident macular edema (P = 0.045) and clinically evident radiation maculopathy (P = 0.040) in the bevacizumab group compared with controls. CONCLUSIONS: Patients receiving intravitreal bevacizumab injection every 4 months after plaque radiotherapy for uveal melanoma demonstrated OCT-evident macular edema, clinically evident radiation maculopathy, moderate vision loss, and poor visual acuity less frequently over a period of 2 years than patients not receiving the injections.

Iris melanoma management with iodine-125 plaque radiotherapy in 144 patients: impact of melanoma-related glaucoma on outcomes.

PURPOSE: To evaluate the outcomes of iris melanoma managed with plaque radiotherapy on the basis of the initial presence or absence of glaucoma. DESIGN: Retrospective, comparative case series. PARTICIPANTS: A total of 144 patients. INTERVENTION: Custom-designed iodine-125 plaque radiotherapy delivering planned 8000 cGy to melanoma apex using transcorneal application. MAIN OUTCOME MEASURES: Tumor control and treatment-related complications. RESULTS: Of 144 patients with iris melanoma, glaucoma was present at the initial visit in 58 (40%). Causes of elevated intraocular pressure included angle infiltration by melanoma in 50 patients (86%), angle neovascularization in 4 patients (7%), and hyphema in 4 patients (7%). At presentation, the eyes displaying iris melanoma with glaucoma (vs. without glaucoma) were statistically more likely to display angle tumor (66% vs. 43%), with minimal thickness (1.9 vs. 2.9 mm), and melanoma seeding in iris stroma (7 vs. 3 clock hours) and angle (5 vs. 2 clock hours). Plaque radiotherapy was performed in all cases. Kaplan-Meier estimates at 7 years post-treatment revealed no statistical differences in outcomes of local recurrence (14% vs. 15%), enucleation (14% vs. 11%), or metastasis (2% vs. 0%) comparing eyes with and without glaucoma. Of the entire group, multivariate analysis for factors predictive of recurrence included partial (vs. complete) anterior segment irradiation and postradiotherapy glaucoma. Factors related to enucleation included diabetes mellitus, poor initial visual acuity, higher radiation dose to tumor apex, and tumor recurrence. There were no factors predictive of metastasis. CONCLUSIONS: Iodine-125 plaque radiotherapy provides adequate tumor control for iris melanoma with a low metastatic potential of 1% at 7 years. Iris melanoma with secondary glaucoma showed a statistically significant greater likelihood of flat tumor with iris and angle seeding and no difference in outcomes compared with eyes without glaucoma. FINANCIAL DISCLOSURE(S): The author(s) have no proprietary or commercial interest in any materials discussed in this article.

Minimal exposure (one or two cycles) of intra-arterial chemotherapy in the management of retinoblastoma.

PURPOSE: To assess the efficacy of less than 3 cycles of intra-arterial chemotherapy (IAC) for retinoblastoma. DESIGN: Retrospective, nonrandomized, interventional case series. PARTICIPANTS: Eight patients. INTERVENTION: Intra-arterial chemotherapy. MAIN OUTCOME MEASURES: Tumor control and globe salvage. RESULTS: Eight patients received fewer than 3 cycles of IAC for retinoblastoma because there was complete tumor control with no residual viable tumor (n = 7) or poor response (n = 1) with little hope that further therapy would benefit the patient. In 3 cases, additional vascular compromise precluded further IAC. The treatment was primary in 6 cases and secondary after failure of other treatment in 2 cases. The 8 eyes were classified (International Classification of Retinoblastoma) as group C (n = 2), group D (n = 3), group E (n = 1), and secondary treatment (n = 2). At initial examination, the main tumor showed a mean basal diameter of 16 mm, a thickness of 8.6 mm, vitreous seeds (n = 2), subretinal seeds (n = 6), and iris neovascularization (n = 1). Three patients were treated with a single cycle of IAC, and 5 patients were treated with 2 cycles of IAC. After IAC, complete tumor response was found in 7 eyes (88%) and partial response was found in 1 eye (13%). Over a mean of 13 months follow-up, there was intraretinal tumor recurrence (n = 1), subretinal seed recurrence (n = 1), and no case of vitreous seed recurrence. Globe salvage was achieved in 2 of 2 group C eyes (100%), 3 of 3 group D eyes (100%), 0 of 1 group E eye (0%), and 1 of 2 secondary treatment eyes (50%). Globe salvage was achieved in 6 of 8 eyes (75%), and 2 of 8 eyes (25%) required enucleation. CONCLUSIONS: One or 2 cycles of IAC can be sufficient for selected eyes with group C or D retinoblastoma, with remarkable tumor control. FINANCIAL DISCLOSURE(S): The author(s) have no proprietary or commercial interest in any materials discussed in this article.

Clinical survey of 3680 iris tumors based on patient age at presentation.

OBJECTIVE: To report the spectrum of iris lesions based on patient age at presentation. DESIGN: Retrospective, nonrandomized, single-center case series. PARTICIPANTS: We included 3680 iris tumors in 3451 patients. METHODS: Chart review. MAIN OUTCOME MEASURES: Diagnostic category based on age. RESULTS: The mean age at presentation was 48 years and there were 449 (12%) tumors in children (≤20 years), 788 (21%) in young adults (21-40 years), 1308 (36%) in mid adults (41-60 years), and 1135 (31%) in senior adults (>60 years). Of 3680 tumors, the diagnostic category was cystic (n = 768; 21%) or solid (n = 2912; 79%). The cystic tumors originated from iris pigment epithelium (IPE; n = 672; 18%) or iris stroma (n = 96; 3%). The solid tumors included melanocytic (n = 2510; 68%) and nonmelanocytic (n = 402; 11%). The melanocytic tumors comprised nevus (n = 1503; 60%), melanocytoma (n = 68; 3%), melanoma (n = 645; 26%), and melanocytosis (n = 64; 3%). Of 2510 melanocytic tumors, the first and second most common diagnoses by age (children, young adult, mid adult, senior adult) were nevus (53%, 57%, 63%, and 63%, respectively) and melanoma (17%, 27%, 26%, and 27%, respectively). The nonmelanocytic tumors included categories of choristomatous (n = 4; <1%), vascular (n = 57; 2%), fibrous (n = 2; <1%), neural (n = 3; <1%), myogenic (n = 2;, <1%), epithelial (n = 35; 1%), xanthomatous (n = 8; <1%), metastasis (n = 67; 2%), lymphoid (n = 12; <1%), leukemic (n = 2; <1%), secondary (n = 12; <1%), and nonneoplastic simulators (n = 198; 5%). The median age (in years) at diagnosis included cystic (39), melanocytic (52), choristomatous (0.7), vascular (56), fibrous (53), neural (8), myogenic (42), epithelial (63), xanthomatous (1.9), metastasis (60), lymphoid (57), leukemic (25.5), secondary (59), and nonneoplastic simulators (49). Overall, the 3 most common specific diagnoses (children, young adult, mid adult, senior adult) were nevus (25%, 36%, 47%, and 47%, respectively), IPE cyst (28%, 30%, 15%, and 14%, respectively), and melanoma (8%, 16%, 20%, and 19%, respectively). CONCLUSIONS: In an ocular oncology practice, the spectrum of iris tumors includes cystic (21%) and solid (79%) tumors. The solid tumors were melanocytic (68%) or nonmelanocytic (11%). At all ages, the most common specific diagnoses were nevus (42%), IPE cyst (19%), and melanoma (17%).

Intravenous and intra-arterial chemotherapy for retinoblastoma: what have we learned?

PURPOSE OF REVIEW: To review the recent literature on two methods of chemotherapy for retinoblastoma using intravenous versus intra-arterial route. RECENT FINDINGS: In 1996, the era of intravenous chemotherapy (chemoreduction) for retinoblastoma was introduced with major centers providing published information on impressive tumor control, without the need for external beam radiotherapy or enucleation. Later reports heralded continued impressive long-term control, minimal systemic toxicities, likely prevention of pinealoblastoma (trilateral retinoblastoma), and reduction in numbers of germline mutation second cancers. There is no reported ophthalmic toxicity and no evidence of reduction in fertility with chemoreduction. In 2011, the era of intra-arterial chemotherapy was announced with several studies and three conflicting editorials in the literature. This technique requires a catheterization through the arterial tree from the femoral artery into the ophthalmic artery. Outstanding tumor control is achieved with only three cycles, but more-than-expected ocular ischemic events have been noted. Further improvements in this technique could minimize complications. SUMMARY: Both intravenous and intra-arterial chemotherapy are powerful methods for retinoblastoma control. In addition to tumor control, intravenous chemotherapy protects from systemic metastasis and pinealoblastoma, minimizes long-term second cancers, and has few systemic and no ocular toxicities. Intra-arterial chemotherapy provides excellent tumor control for slightly more advanced eyes with retinoblastoma and, in addition, can be used to treat eyes that fail other methods. However, local ocular toxicities can be vision-threatening and long-term systemic toxicities are not yet understood.

Sector laser photocoagulation for the prevention of macular edema after plaque radiotherapy for uveal melanoma: a pilot study.

OBJECTIVE: To investigate the role of sector laser photocoagulation for prevention of macular edema after plaque radiotherapy for uveal melanoma. METHODS: Noncomparative, pilot interventional case series. The main outcome measure was optical coherence tomography-evident macular edema. RESULTS: A total of 29 patients had sector laser photocoagulation (sector panretinal photocoagulation) and sub-Tenon triamcinolone injection. The median tumor thickness and base was 3.3 mm and 10.0 mm. The median radiation dose and rate to the macula was 2,944 cGy and 31.0 cGy/hour. At the 12-month and 24-months follow-up, cystoid macular edema was found in 17% and 24% of the sector panretinal photocoagulation group. There were no major side effects registered. CONCLUSION: Sector panretinal photocoagulation in combination with sub-Tenon triamcinolone appears to show potential as a safe and beneficial intervention for the prevention of macular edema after plaque radiotherapy for uveal melanoma in this series.

Effect of intraarterial chemotherapy on retinoblastoma-induced retinal detachment.

PURPOSE: To study the effect of intraarterial chemotherapy (IAC) on retinoblastoma-induced retinal detachment. METHODS: Retrospective, noncomparative, interventional case series including 15 patients, with intraarterial (ophthalmic artery) chemotherapy as the intervention. Resolution of retinal detachment was the main outcome measure. RESULTS: Of 15 patients with retinoblastoma-induced retinal detachment managed with IAC, 7 (47%) presented with total retinal detachment and 8 (53%) with partial detachment. The eyes were classified as International Classification of Retinoblastoma group C (n = 2, 13%), group D (n = 4, 27%), and group E (n = 5, 33%). There were 4 eyes (27%) that received IAC as secondary treatment after the failure of other treatment methods. After IAC, all tumors showed regression. Of the 7 eyes with initial total retinal detachment, complete resolution of fluid was found in 3 (43%) and partial resolution in 4 (57%). Of the 8 eyes with initial partial retinal detachment, complete resolution of subretinal fluid was found in all 8 (100%), but 1 eye later developed recurrent subretinal fluid. The mean interval to complete resolution of subretinal fluid was 2 months. Globe salvage was achieved in 5 of 7 eyes (71%) with total retinal detachment and 6 of 8 eyes (75%) with partial retinal detachment. CONCLUSION: Eyes with retinoblastoma-induced total or partial retinal detachment showed complete resolution of detachment after IAC in 43% and 100%, respectively.

Prognosis of uveal melanoma in 500 cases using genetic testing of fine-needle aspiration biopsy specimens.

PURPOSE: To determine the relationship between monosomy 3 and incidence of metastasis after genetic testing of uveal melanoma using fine-needle aspiration biopsy (FNAB). DESIGN: Noncomparative retrospective case series. PARTICIPANTS: Five hundred patients. METHODS: Fine-needle aspiration biopsy was performed intraoperatively immediately before plaque radiotherapy. The specimen underwent genetic analysis using DNA amplification and microsatellite assay. Systemic follow-up was obtained regarding melanoma-related metastasis. MAIN OUTCOME MEASURES: Presence of chromosome 3 monosomy (loss of heterozygosity) and occurrence of melanoma metastasis. RESULTS: Disomy 3 was found in 241 melanomas (48%), partial monosomy 3 was found in 133 melanomas (27%), and complete monosomy 3 was found in 126 melanomas (25%). The cumulative probability for metastasis by 3 years was 2.6% for disomy 3, 5.3% for partial monosomy 3 (equivocal monosomy 3), and 24.0% for complete monosomy 3. At 3 years, for tumors with disomy 3, the cumulative probability of metastasis was 0% for small (0-3 mm thickness), 1.4% for medium (3.1-8 mm thickness), and 23.1% for large (>8 mm thickness) melanomas. At 3 years, for tumors with partial monosomy 3, the cumulative probability of metastasis was 4.5% for small, 6.9% for medium, and [insufficient numbers] for large melanomas. At 3 years, for tumors with complete monosomy 3, the cumulative probability of metastasis was 0% for small, 24.4% for medium, and 57.5% for large melanomas. The most important factors predictive of partial or complete monosomy 3 included increasing tumor thickness (P = 0.001) and increasing distance to optic disc (P = 0.002). CONCLUSIONS: According to FNAB results, patients with uveal melanoma demonstrating complete monosomy 3 have substantially poorer prognosis at 3 years than those with partial monosomy 3 or disomy 3. Patients with partial monosomy 3 do not significantly differ in outcome from those with disomy 3.

Ocular surface squamous neoplasia (squamous cell carcinoma) of the socket: management of extensive tumors with interferon.

PURPOSE: To describe the clinical features and management of extensive ocular surface squamous neoplasia (OSSN) (squamous cell carcinoma) of the socket. METHODS: Retrospective interventional case series. Interferon α 2b (IFNa2b) eye drops (1 million units/cc) 4 times daily and IFNa2b sublesional injection (5 million units/0.5cc to 8 million units/0.8 cc) were delivered for tumor control. Participants were 3 patients with ocular prosthesis who developed extensive socket OSSN. Tumor control was graded as complete regression, partial regression, or no regression. RESULTS: OSSN was detected in the socket at age 60, 43, and 20 years in patients who had worn ophthalmic prostheses for 54, 26, and 13 years, respectively. The patients had chronic discharge and irritation (n = 3) managed with intermittent topical corticosteroids (n = 2). There were no predisposing factors of cigarette exposure, radiation exposure, eczema, systemic immune suppression, or organ transplantation. The prosthesis fit well with nonirritative edges. At presentation, OSSN was subtle (n = 3), vascular (n = 3), and multifocal (n = 3), with largest lesions or confluence of lesions measuring 20, 25, and 20 mm, respectively. The tumors involved the tarsal (n = 3), bulbar (n = 2), and forniceal (n = 2) surfaces. All patients were treated with topical and injection IFNa2b, with complete regression achieved in 2 cases (at 1 months and 20 months) and partial regression in one case (at 9 months). All patients continue on chronic maintenance IFNa2b topically. There were no recurrences, and IFNa2b injection side effects of nausea and chills were minor, lasting 1 day. No patient required surgical removal of tumors from the socket and no patient required exenteration. CONCLUSIONS: Patients wearing ophthalmic prosthesis over a socket should be monitored for the development of OSSN. Combined topical and injection IFNa2b could represent a potentially effective therapy for this condition.

Topical imiquimod for periocular lentigo maligna.

PURPOSE: To evaluate the efficacy of topical imiquimod 5%, a local immune response modifier, in the treatment of periocular lentigo maligna. DESIGN: Retrospective, interventional case series. PARTICIPANTS: Five consecutive patients with biopsy-proven periocular lentigo maligna. METHODS: Periocular lentigo maligna was treated with topical imiquimod 5%. The clinical features, treatment schedule, response to treatment, and complications were analyzed retrospectively. MAIN OUTCOME MEASURES: Response to treatment and complications. RESULTS: The mean patient age was 73 years. The anatomic location of lentigo maligna was the medial canthal area in 2 patients, the lateral canthal area in 1 patient, and the lower eyelid in 2 patients. Topical imiquimod 5% was used for 5 days per week in 3 patients and for 7 days per week in 2 patients. The medication was placed only on the skin and not the globe. The mean duration of treatment was 9 months (range, 1-14 months). Lentigo maligna partially resolved in 3 patients and completely resolved in 2 patients. The most common side effects included localized erythema and discomfort (n = 4), swelling (n = 3), and cutaneous excoriation (n = 2). There were no patients with toxicity to the conjunctiva, cornea, or globe. Treatment was discontinued in 2 patients (one temporarily and the other permanently) because of intolerable local side effects of discomfort, redness, swelling, and cutaneous excoriation. There was no recurrence of lentigo maligna in those with complete or partial response (mean follow-up, 20 months). CONCLUSIONS: Periocular lentigo maligna seems to respond to topical imiquimod 5% treatment. Topical imiquimod 5% treatment for periocular lentigo melanoma deserves further study. FINANCIAL DISCLOSURE(S): The author(s) have no proprietary or commercial interest in any materials discussed in this article.

Single-agent, minimal dose, minimal exposure intraarterial chemotherapy for retinoblastoma in a 4-month-old infant.

PURPOSE: To document minimal dose and minimal exposure of chemotherapy for unilateral retinoblastoma. METHODS: A 4-month-old infant developed leukocoria in the right eye and was found to have unilateral sporadic retinoblastoma. RESULTS: The right eye was classified as Group D retinoblastoma, with a single large tumor, moderate subretinal seeding, and total retinal detachment. The retinoblastoma measured 20 mm in basal dimension and 13 mm in ultrasonographic thickness. Options of enucleation, intravenous chemotherapy, and intraarterial chemotherapy were offered, but the latter was chosen because of anticipated, rapid, and more complete response with intraarterial rather than intravenous chemotherapy. After using low dose (3 mg) of single-agent melphalan delivered over 30 minutes into the ostium of the ophthalmic artery of the 4-month-old infant, a complete response with Type 1 regression (complete calcification) of the mass and resolution of all subretinal fluid was found. A second similar dose was delivered to ensure remission of all seeds and tumor with stable findings. Further chemotherapy was stopped. On 6 months of follow-up, the child displayed complete tumor control with 2 cycles of lowest dose (3 mg) intraarterial melphalan. There were no complications. CONCLUSION: In infants younger than 6 months, low dose of only 3-mg single-agent melphalan could be sufficient to control retinoblastoma with minimal exposure.

Genomic profile of 320 uveal melanoma cases: chromosome 8p-loss and metastatic outcome.

PURPOSE: Uveal melanoma (UM) was a fatal malignancy in 40% to 50% of cases. The aim of this study is to evaluate the independent contributions of chromosome 1, 3, 6, and 8 abnormalities for prognostication of metastasis, and to define multichromosome copy number aberration (CNA) signatures that can be used to evaluate risk. METHODS: A series of 320 UM were analyzed for chromosome 1, 3, 6, and 8 abnormalities using whole genome single-nucleotide polymorphism arrays. Results for changes in six chromosomal regions were analyzed using univariate and multivariate Cox proportional hazard modeling to identify significant predictors of metastasis and CNA signatures. RESULTS: Univariate Cox analysis indicated that losses of chromosome 3, 1p, 6q, and 8p and gain of 8q, as well as sex, source of tumor tissue (fine-needle aspiration biopsy [FNAB] compared with tumor from an enucleated eye), tumor basal diameter and height, and ciliary body involvement were all significant predictors of poor metastatic outcome. In the multivariate analysis, loss of chromosome 3 and 8p remained significant after adjusting for the effects of all other variables, as did sex, tissue source, and basal diameter. Multivariate analysis of the joint effects of changes in the six chromosomal regions showed that six signatures, including chromosome 3-loss, 1p-loss, 8p-loss, and/or 8q-gain had hazard ratios (HR) ranging from 7.90 to 37.25. CONCLUSIONS: In UM, tumor size and location, tissue source, and sex were all significantly associated with increased metastasis. In addition, chromosome 3-loss and 8p-loss were found to be independent predictors of poor metastatic outcome and CNA signatures were identified that can add a specific HR value for classification of risk categories.

Combined hamartoma of the retina and retinal pigment epithelium in a child with branchial cleft cysts.

An otherwise healthy 15-month-old boy presented with benign branchial cleft cysts and combined hamartoma of the retina and retinal pigment epithelium. This association was previously only reported in a patient with branchio-oculo-facial syndrome. Although uncommon, this could represent multifocal sites of fetus craniofacial malformation.

Pearls and pitfalls of intraarterial chemotherapy for retinoblastoma.

Retinoblastoma is a deadly eye cancer in children, leading to death in 50%-70% of children in undeveloped nations who are diagnosed with it. This malignancy is the most common intraocular tumor in childhood worldwide. The good prognosis in developed nations is related to early detection and advanced treatments. With the advent of intraarterial chemotherapy, neurosurgeons have taken a central role in the treatment of this pediatric condition. Intraarterial chemotherapy is a novel treatment for retinoblastoma whereby chemotherapeutic agents are precisely delivered into the ophthalmic artery, minimizing systemic toxicity. This procedure has shown impressive results and has allowed a dramatic decrease in the rate of enucleation (eye removal) in advanced and refractory retinoblastoma. Recent reports have raised some concerns about the risk of ocular vasculopathy, radiation-related toxicity, and the potential for metastatic disease after intraarterial chemotherapy. In the authors' experience of more than 3 years, tumor control is excellent with globe salvage at 67% and vascular events less than 5%, mostly related to improvement in technique. The role of this novel approach in the management of retinoblastoma has yet to be defined. As more centers are adopting the technique, the topic will decidedly become the focus of intensive future research. In this paper, the authors review and discuss current data regarding intraarterial chemotherapy for retinoblastoma.

Safety of pars plana vitrectomy in eyes with plaque-irradiated posterior uveal melanoma.

OBJECTIVE: To determine the long-term safety of pars plana vitrectomy (PPV) in eyes with plaque-irradiated posterior uveal melanoma. METHODS: In this retrospective case series, patients with plaque-irradiated posterior uveal melanoma subsequently underwent PPV for vitreous hemorrhage. The main outcome measures are the rates of intraocular melanoma dissemination, extrascleral extension of melanoma, local melanoma recurrence, and systemic melanoma metastasis after PPV. RESULTS: Forty-seven eyes of 47 patients underwent PPV for vitreous hemorrhage after iodine 125-labeled plaque radiotherapy for choroidal melanoma. The mean interval between the onset of vitreous hemorrhage and PPV was 13 (median, 10; range, 0-52) months. The mean time from PPV to last follow-up was 5 (range, 0.5-16) years. There were no cases of intraocular melanoma dissemination or extrascleral extension of melanoma. One patient (2%) developed local choroidal melanoma recurrence (2 years after PPV and 5 years after initial plaque radiotherapy) and was successfully managed with transpupillary thermotherapy. Systemic melanoma metastasis occurred in 4 patients (9%) during a mean interval of 5 years after plaque radiotherapy. During follow-up, 43 patients (91%) were alive without systemic metastasis and 4 patients (9%) were alive with metastasis. CONCLUSION: Management of vitreous hemorrhage by PPV in eyes with previously irradiated uveal melanoma appears to be safe and without increased risk for intraocular, local, orbital, or systemic dissemination of the tumor.

Intravenous chemoreduction or intra-arterial chemotherapy for cavitary retinoblastoma: long-term results.

OBJECTIVE: To assess the long-term results of chemotherapy for cavitary retinoblastoma. METHODS: Retrospective, nonrandomized, interventional case series of 26 cavitary retinoblastomas in 25 eyes of 24 patients. Retinoblastomas were treated with intravenous chemoreduction and/or intra-arterial chemotherapy. Main outcome measures included tumor control, globe salvage, and metastasis. RESULTS: Of 24 patients with cavitary retinoblastoma, the mean age at diagnosis was 16 months. The mean number of cavitary tumors per eye was 1 (median, 1; range, 1-2), with a mean tumor basal diameter of 13 (median, 13; range, 7-24) mm and mean tumor thickness of 7 (median, 6; range, 3-17) mm. The mean number of cavities per tumor was 2 (median, 2; range, 1-5), with a mean cavity diameter of 3 (median, 2; range, 1-10) mm. Related features included vitreous seeds in 7 tumors (27%), subretinal seeds in 6 (23%), and subretinal fluid in 13 (50%). Intravenous chemoreduction was used in 23 tumors (88%); intra-arterial chemotherapy, in 2 (8%); and both, in 1 (4%). After treatment, the mean reduction in tumor base was 22% and mean reduction in tumor thickness was 29%. Despite minimal reduction, tumor recurrence was noted in only 1 eye (4%), globe salvage was achieved in 22 (88%), and there were no cases of metastasis or death during 49 (range, 6-189) months of follow-up. CONCLUSION: Despite minimal visible tumor response to chemotherapy, cavitary retinoblastoma displays relatively stable long-term results.

Acute exudative paraneoplastic polymorphous vitelliform maculopathy in five cases.

BACKGROUND AND OBJECTIVE: To evaluate clinical features, course, and outcome of patients with acute exudative paraneoplastic polymorphous vitelliform maculopathy (AEPPVM). PATIENTS AND METHODS: Retrospective case series of 5 patients. RESULTS: There were 3 males and 2 females, with a median age of 74 years. The primary neoplasms were cutaneous melanoma (n = 2), choroidal melanoma (n = 1), lung adenocarcinoma (n = 1), and lung plus breast adenocarcinoma (n = 1). The mean interval between the diagnosis of the primary neoplasm and the diagnosis of AEPPVM was 42 months. The presenting symptom was blurred vision in all cases. Ophthalmoscopy disclosed multifocal localized shallow serous detachments of the post-equatorial neurosensory retina with yellow-white subretinal debris confirmed by optical coherence tomography (OCT). There was a mean of 21 individual sites of detachment per eye, each measuring a mean of approximately 0.8 millimeter in diameter. Fundus autofluorescence depicted hyperautofluorescence corresponding to the detachments. After mean follow-up of 5 months, three patients had died of metastases. Of the two survivors, one showed resolution of lesions and the other was unchanged. CONCLUSION: AEPPVM is a paraneoplastic retinopathy found in patients with metastatic melanoma or carcinoma. The most salient feature is reduced visual acuity from multifocal shallow retinal detachments less than 1-mm diameter, best depicted on OCT.