Case of the Month

Edited by Robert N. Johnson, MD

Case #110, August, 2018

Presented by:

Michelle Peng, MD

A 57-year-old Caucasian man with recent onset of blurred vision in his right eye.

Figure 1: Color montage of the right eye. Note the serous macular detachment. there is a grayish elevated lesion superior to the fovea.

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Figures 2A-C: SD-OCT of the right eye. Figure A is a horizontal scan through the central macula showing the serous detachment. A horizontal (Figure 2B) and vertical (Figure 2C) through the elevated lesion is show showing a choroidal mass with a mostly smooth surface.

Case History

A 57-year-old Caucasian presented with the complaint of gradual decrease in central vision in the right eye of one week’s duration.  His past ocular history was unremarkable. He was HIV positive and had a prior history of cutaneous melanoma.

On examination, visual acuity was 20/125 in the right eye and 20/32 in the left eye. The anterior segment examination was unremarkable. Dilated examination of the right eye revealed a serous macular detachment and a lightly pigmented, elevated choroidal mass in the superior macula (Figure 1). The left eye was unremarkable. Ocular coherence tomography displayed a thickened choroidal mass with subretinal fluid (Figure 2A-C). Fluorescein angiography revealed punctate areas of hyperfluorescence over the lesion (Figure 3). On ultrasonography the mass measured 1.8mm (Figure 4).

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Figures 3A-C: Fluorescein angiogram of the right eye. Note the multiple areas of pinpoint hyperfluorescence over the surface of the choroidal lesion. In the later phases, there is mild leakage.

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Figure 4: B-scan ultrasound showing a solid lesion. It measured 1.8mm in thickness

What is your Diagnosis?

Differential Diagnosis

Choroidal nevus, Hemorrhagic retinal pigment epithelial detachment, choroidal neovascularization, choroidal granuloma, Congenital hypertrophy of the retinal pigment epithelium, Retinal pigment hyperplasia, Choroidal metastasis, Choroidal hemangioma

 

Additional History and Diagnosis

The patient had a history of HIV and treated cutaneous melanoma of the left occiput status post prior lymph node dissection 13 years earlier. He then developed a recurrence 10 years after initial treatment with positive neck lymph nodes on biopsy (3 years prior to onset of vison problems). Approximately 3 years later routine surveillance imaging revealed two liver lesions, which were biopsy positive for melanoma, lung lesions, and lumbar spine lesions. He then underwent radiation and was started on pembrolizumab. Given his extensive history of cutaneous melanoma and recent findings of metastatic disease, there was high suspicion for a choroidal metastasis. After 3 treatments of pembrolizumab, little change in the metastatic lesion was noted. The patient was switched to  dabrafenib and trametinib therapy with a reduction in size of choroidal mass and resolution in serous fluid after three months of treatment.

 

Discussion

Choroidal metastasis from cutaneous melanoma is uncommon. Cutaneous melanomas account for less than 5% of all metastases to the eye and orbit.2 A large review noted that upon diagnosis the majority of patients are between the ages of 30 to 70 years of age, with relatively equal predilection for men and women.3

Cutaneous malignant melanomas have been seen to involve all aspects of the eye and orbit.3 The uveal tract is more commonly affected than the retina.4 It is posited that blood flow, variations in permeability of retinal vascular endothelium, and inhibitory factors are involved in the rarity of metastatic cancer to the retina compared to the uveal tract.1 Melanomas may also metastasize to the vitreous and present as spherical clumps of brown melanoma cells.5 The presence of these cells may be accompanied by fluffy dendritic gray-brown infiltrates within the peripapillary nerve fiber layer. There may also be irregular plaques of melanoma cells within the retina.1

The diagnosis of choroidal metastasis relies on clinical history and examination. Patients often carry a known diagnosis of a primary cutaneous lesion with visceral metastasis, but at times findings in the eye can be the first sign of disease.5 Clinically metastatic cutaneous melanomas tend to progress more rapidly and tend to be multifocal. In the setting of bilateral, multifocal, diffuse ocular melanoma, cutaneous disease must be highly considered.3

Clinical suspicion with appropriate history is considered sufficient for diagnosis. Fluorescein angiogram findings can be variable and demonstrate early hyperfluorescence, a mottled pattern in midvenous phase, and tumor staining in late frames (similar to what was seen in our case).6 Choroidal metastases tend to have a lumpy contour on ultrasonography with medium to high internal reflectivity and an irregular internal structure. Biopsy may be beneficial in patients without extraocular metastasis to help support whether the tumor might be of melanocytic origin. Diagnosis is important to determine proper treatment. Early detection improves prognosis particularly in cases with stage IV disease.3

Treatment of metastatic melanoma is systemic, with close observation of the intraocular lesion(s). Those with suspected metastatic melanoma should be concurrently managed with a medical oncologist. Plaque brachytherapy may be employed in cases where a solitary tumor presents a threat to vision. Pars plana vitrectomy may be considered in cases of vitreous involvement.3

Take Home Points

  • Ocular metastasis from cutaneous melanoma represents a rare entity that manifests more commonly in the uveal tract, but can affect any part of the eye and its surrounding tissues.
  • Diagnosis is based on supplemental clinical history and examination with an emphasis on identifying primary versus metastatic disease to aid in directing the course of treatment.
  • Treatment is primarily systemic with close monitoring of the ocular lesion.

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References

  1. Agarwal A. Metastatic cutaneous melanoma to the retina and vitreous. In: Agarwal, A. Gass’ Atlas of Macular Diseases. Fifth ed. Elsevier:1166-1167.
  2. Goldberg RA, Rootman J, Cline RA: Tumors metastatic to the orbit: a changing picture. Surv Ophthalmol 1990;35:1-24.
  3. Rosenberg C and Finger PT. Cutaneous malignant melanoma metastatic to the eye, lids, and orbit. Survey of Ophthalmology 2008;53:187-202.
  4. Riffenburgh RS. Metastatic malignant melanoma to the retina. Arch Ophthalmol 1961;66:487-9.
  5. Cole EL, Zakov ZN, Meisler DM, et al: Cutaneous malignant melanoma metastatic to the vitreous. Arch Ophthalmol 1986;104:98-101.
  6. Letson AD, Davidorf RH. Bilateral retinal metastases from cutaneous malignant melanoma. Arch Ophthalmol 1982;100:605-7.

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