Cutaneous melanoma metastatic to the orbit: review of 15 cases.

PURPOSE: To describe the clinical features, management, and outcomes of 15 patients with cutaneous melanoma metastatic to the orbit. The authors review emerging treatments for metastatic melanoma and their ocular implications. METHODS: Retrospective chart review of 15 patients with orbital metastasis from cutaneous melanoma. RESULTS: At presentation of the orbital metastasis, systemic metastatic cutaneous melanoma was present in 13 (87%) patients. The mean interval from diagnosis of cutaneous melanoma to orbital metastasis was 40 months (median, 37 months; range, 0-117 months). The most common presenting signs were dysmotility (63%), proptosis (56%), and blepharoptosis (19%). Four patients (25%) presented with pain. Metastasis involved extraocular muscle in 6 orbits (35%), intraconal space in 4 (24%), extraconal space in 7 (41%), and lacrimal sac in 1 (6%). The tumor was unifocal in all cases, unilateral in 13 patients (87%), and bilateral in 2 (13%). The mean tumor basal dimension was 20 × 20 mm and mean thickness was 16 mm. Treatments included complete surgical excision in 1 patient (6%), external beam radiotherapy (EBRT) in 7 (47%), systemic chemotherapy in 8 (53%), and immunotherapy in 5 (33%). Orbital tumor control was achieved in 2 orbits (18%) following focal therapy alone (excision or EBRT), 4 (36%) following systemic therapy alone (chemotherapy or immunotherapy), and 3 (27%) following combination focal plus systemic therapy. Three patients required exenteration. Survival rates at 1 year/2 years were 100%/0% following focal therapy, 50%/25% following systemic therapy, and 100%/66% following combination therapy. CONCLUSIONS: Cutaneous melanoma metastatic to the orbit tends to involve muscle (35%) or intraconal soft tissue (24%) as a painless (75%), circumscribed (87%) mass. Treatment with systemic chemotherapy and/or immunotherapy resulted in orbital tumor control in 80% of cases. Overall survival was 25.1 months.

The seasick choroid: a finding on enhanced depth imaging spectral-domain optical coherence tomography of choroidal lymphoma.

PURPOSE: To describe previously unreported imaging features of choroidal lymphoma using enhanced depth imaging optical coherence tomography (OCT). METHODS: Enhanced depth imaging OCT was performed before and after the therapy. RESULTS: A 32-year-old white man with a 4-month history of blurred vision in the right eye was found to have a macular fold. There was no visible intraocular tumor. There were no signs of anterior segment inflammation, vascular abnormalities, or infiltrative disease. Visual acuity was 20/150 in the right eye and 20/20 in the left eye. Enhanced depth imaging OCT demonstrated a macular retinal fold and marked thickening of the choroid with striking choroidal surface undulation and folds imparting an appearance similar to a "sea storm" (seasick appearance). Deep choroidal structures could not be visualized, and the sclerochoroidal interface could not be identified. Overlying subretinal fluid and intraretinal fluid was noted. Ultrasonography demonstrated diffuse, relatively smooth thickening of the choroid (4.0-mm thickness) with minor extraocular hypoechoic area. Based on these findings, choroidal lymphoid proliferation was suspected, and fine-needle aspiration biopsy confirmed B-cell lymphoma. Results of systemic evaluation were unremarkable. After external beam radiotherapy with dose of 40 Gy, visual acuity returned promptly to 20/40 and the lymphoid infiltration resolved with flattening of the macular fold and resolution of subretinal and intraretinal fluids. The enhanced depth imaging OCT returned to a more normal appearance with the resolution of the retinal fold and reduction of the choroidal mass with retinal pigment epithelial-choroidal surface features to a "calm sea" appearance. CONCLUSION: Enhanced depth imaging OCT is a useful tool for subclinical monitoring of choroidal infiltration from lymphoma before and after therapy.

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.