Figure. A 30-year-old man with a small choroidal melanoma had overlying orange pigment sediment before treatment (A), with slight reduction in orange pigment at 4 (B) and 8 (C) months after plaque radiotherapy. Autofluorescence confirmed the lipofuscin at the initial visit (D), with reduction at 4 (E) and 8 (F) months after radiotherapy. Optical coherence tomography showed subretinal fluid and lipofuscin debris at the initial visit (G), and the fluid showed resorption at 4 months (H) but persistent subretinal debris at 4 (H) and 8 (I) months after radiotherapy.
Hashmi F, Rojanaporn D, Kaliki S, Shields CL. Orange Pigment Sediment Overlying Small Choroidal Melanoma. Arch Ophthalmol. 2012;130(7):937–939. doi:10.1001/archophthalmol.2011.1907
Author Affiliations: Ocular Oncology Service, Wills Eye Institute, Thomas Jefferson University, Philadelphia, Pennsylvania.
Lipofuscin-laden macrophage accumulation can occur over benign and malignant choroidal tumors.1 The presence of overlying lipofuscin pigment has been demonstrated with choroidal nevus, choroidal hemangioma, benign reactive lymphoid hyperplasia, intraocular lymphoma, choroidal metastasis, and, most often, choroidal melanoma.2 Lipofuscin pigment typically appears as fine or granular deposits over the surface of a thin choroidal mass. Accretion of lipofuscin layered as sediment is extremely rare. Furthermore, a PubMed search for keywords “lipofuscin,” “sediment,” “orange,” “melanoma,” “nevus,” “choroid,” “uvea,” and “eye” produced no cases of lipofuscin sediment overlying choroidal melanoma and 1 case overlying nevus.3 Herein, we describe a small choroidal melanoma with orange pigment (lipofuscin) sediment.
A 30-year-old man had blurred vision in the left eye for 2 weeks. On examination, visual acuity was 20/20 OD and 20/25 OS. Anterior segment examination findings were unremarkable in both eyes. Fundus examination findings in the right eye were unremarkable. Fundus examination of the left eye revealed a pigmented choroidal mass in the macular area, with a tumor base of 6 mm and a thickness of 2 mm by ultrasonography, consistent with small choroidal melanoma. There was subretinal fluid with profound lipofuscin appearing as dispersed orange pigment overlying the melanoma and forming subretinal orange sediment (Figure). The sediment displayed bright hyperautofluorescence and blocked fluorescein dye on angiography. Optical coherence tomography demonstrated subretinal fluid and confirmed subretinal debris consistent with lipofuscin deposition.
The small choroidal melanoma was treated with iodine 125 plaque radiotherapy. At 4 months' follow-up, visual acuity was 20/20 OS, subretinal fluid had resolved, and orange pigment had reduced (Figure). At 8 months' follow-up, visual acuity was 20/20 OS, the melanoma had regressed to a thickness of 1.2 mm, and there was minimal residual orange pigment clinically and by autofluorescence.
Histochemical and electron microscopic studies have characterized orange pigment in the subretinal space overlying choroidal tumors as representing aggregates of macrophages with lipofuscin pigment (periodic acid–Schiff positive) and melanin from damaged retinal pigment epithelium.4 It is speculated that choroidal tumors disrupt overlying retinal pigment epithelium and interfere with lipofuscin metabolism, resulting in orange pigment.
In a clinical and histopathologic study of 107 choroidal tumors,2 lipofuscin pigment was present overlying choroidal melanoma (90 of 100 cases [90%]), choroidal metastasis (5 of 5 cases [100%]), choroidal hemangioma (1 of 1 case [100%]), and benign reactive lymphoid hyperplasia (1 of 1 case [100%]). The color of lipofuscin varies and appears orange over brown tumors and brown over yellow or orange tumors.2 Autofluorescence studies have demonstrated lipofuscin overlying choroidal nevus, choroidal melanoma, choroidal hemangioma, and retinoblastoma.1,2 With regard to choroidal nevus, the presence of orange pigment is a predictor for transformation into melanoma.5
Orange pigment sediment over choroidal melanoma resembles the pseudohypopyon of Best vitelliform macular dystrophy clinically, on fundus autofluorescence, and on optical coherence tomography.6 Orange pigment sediment has been previously reported as orange pigment pseudohypopyon in a single case of choroidal nevus in which the lipofuscin disappeared slowly over 3 years.3 Following publication of that case, further follow-up by one of us (C.L.S.) disclosed eventual growth into melanoma. Following plaque radiotherapy, lipofuscin might increase related to retinal pigment epithelial disturbance,7 but eventually it fades from cell death, as in our case.
Correspondence: Dr C. L. Shields, Ocular Oncology Service, Wills Eye Institute, Thomas Jefferson University, 840 Walnut St, 14th Floor, Philadelphia, PA 19107 (firstname.lastname@example.org).
Author Contributions: Dr Shields had full access to all of the data in the study and takes responsibility for the integrity of the data and accuracy of the data analysis.
Financial Disclosure: None reported.
Funding/Support: This work was supported by the Eye Tumor Research Foundation, Philadelphia, Pennsylvania (Dr Shields).
Role of the Sponsor: The sponsor had no role in the design and conduct of the study; in the collection, analysis, and interpretation of the data; or in the preparation, review, or approval of the manuscript.