Lipid Dominant Toxic Tumor Syndrome

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Objective: To describe clinical features of lipid dominant toxic tumor syndrome as a chronic variant of toxic tumor syndrome and report outcomes following use of intravitreal steroids. Design: Retrospective interventional case series. Subjects, Participants, and/or Controls: Records of thirteen patients who had lipid dominant toxic tumor syndrome following treatment of choroidal melanoma with episcleral plaque brachytherapy (EPB) were reviewed. Methods, Intervention, or Testing: Retrospective case series. Main Outcome Measures: Resolution of Lipid exudates, subfoveal subretinal fluid, cystoid macular edema, exudative detachment. Results: Of the thirteen patients who developed lipid dominant toxic tumor syndrome, 11(85%) had medium-sized melanomas, and 2(15%) small-sized melanomas. Average time to onset following EPB was 22 months (range 3-48 months). 7 patients (54%) were noted to have dyslipidemia. The baseline mean visual acuity at the time of diagnosis of toxic tumor syndrome (TTS) was 50 ETDRS letters (range 10 to 85). Ophthalmic characteristics were lipid exudates centered around the tumor base in 13(100%) patients, subfoveal subretinal fluid in 4(31%) patients and cystoid macular edema (CME) in 2(15%) patients. Exudative detachment was absent in all (100%) patients. Regressed melanoma was present in all (100%) patients. Eight (62%) patients were treated with intravitreal steroids (4mg triamcinolone) while 5 patients (38%) were observed. The response to intravitreal steroid was noted in 7(88%) of the treated patients, with the average time to resolution being 1.9 months. Features characterizing a positive response were reduction in lipid exudates centered around the tumor base (100%), reduction in subfoveal subretinal fluid (100%), and reduction in CME (50%). Cataract development was seen in 10 (83%) and ocular hypertension in 3 patients (23%). Proliferative radiation retinopathy developed in 2(15%) patients, neovascular glaucoma developed in 1(8%) while no patients required enucleation. Conclusions: The lipid dominant toxic tumor syndrome centered around the tumor base that occurs in a radiation responsive tumor could be considered a chronic variant in the spectrum of the toxic tumor syndrome. Intravitreal steroids in selected cases reverses the course of this variant, stabilizing or improving the vision. Our observations would need to be verified through a larger prospective study.