Vitelliform retinopathy in patients with metastatic melanoma is very rare. Only three cases associated with choroidal melanoma,1, 2, 3 five cases with cutaneous melanoma,3, 4, 5 and one case with metastatic melanoma of an unknown primary site2 have been reported yet. Here we describe a patient with metastatic choroidal melanoma and paraneoplastic vitelliform fundus findings.

Case report

A 62-year-old woman presented in July 2005 with gradual loss of vision in her left eye. She had no complaints of photopsias or night blindness but reported variable glare. Her medical history was significant for choroidal melanoma in the right eye that was treated by enucleation in 2003. On examination, best-corrected visual acuity was 20/40 in the left eye. Slit-lamp examination was unremarkable. Ophthalmoscopy showed multiple yellowish areas of serous retinal detachment along the temporal arcades (Figure 1). Fluorescein angiography showed some blocked fluorescence corresponding to the yellowish lesions in the left eye. Optical coherence tomography (OCT) showed a serous pigment epithelial detachment (PED) with thickening of the pigment epithelial layer. Ultrasonography, perimetry, electroretinography (ERG), and electrooculography (EOG) were unremarkable. Antiretinal antibodies, including antirecoverin and antienolase could not be demonstrated using western blot technique and immunohistochemistry.

Figure 1
figure 1

Upper left: colour fundus photograph depicting yellow subretinal exudates. Upper right: early-phase FA; lower-left: late-phase FA, shows subtle blocking of fluorescence corresponding to the yellowish lesions. Lower right: OCT showing PED with no intraretinal or subretinal fluid.

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Systemic work-up including computed tomography (CT) scanning detected the presence of liver and lung masses. The patient did not receive any treatment.

During 20 months of follow-up, the fundus lesions had altered in configuration. Visual acuity decreased to 20/50. ERG examinations remained unremarkable.

The patient died in May 2007.

Comment

The vitelliform retinopathy described in this patient resembles those in the nine cases of metastatic melanoma described in previous works.1, 2, 3, 4, 5 In four cases, both antiretinal antibodies and ERG abnormalities were found.1, 3, 4, 5 In one case, antiretinal antibodies were detected; however, ERG was not performed.4 In the other four cases, neither antibodies nor ERG were tested.2, 3

ERG abnormalities together with antiretinal antibodies suggest a paraneoplastic origin like melanoma-associated retinopathy (MAR). In our patient with no antiretinal antibodies and a normal ERG, MAR syndrome is unlikely.6

The vitelliform lesions of our patient may resemble other disorders with vitelliform lesions like acute exudative polymorphous vitelliform maculopathy7 (AEPVM) or adult vitelliform macular dystrophy8 (AVMD). Both diseases show normal ERG and no antiretinal antibodies. However in contrast to our case, AVMD patients show a solitary subretinal lesion in the fovea. AEPVM consists of bilateral vitelliform lesions and visual loss, which resolve within several months, accompanied by subnormal ERG and EOG. Neither one has associations with ocular or systemic malignities.

Fundus configuration as well as the course of the mentioned diseases are quite different from that of our patient.

Although our patient had a normal ERG and no antiretinal antibodies, the association of vitelliform lesions with metastatic choroidal melanoma suggests a paraneoplastic origin. The pathogenesis of paraneoplastic retinopathy is poorly understood. Further studies are required for a better understanding of the aetiology.