A clinically challenging diagnosis of adenoma of the retinal pigment epithelium presenting with clinical features of choroidal hemangioma

Abstract

BackgroundAdenoma of the retinal pigment epithelium (RPE) is a rare intraocular tumor that can simulate other pigmented tumors such as choroidal melanoma. We report a case of non-pigmented adenoma of the RPE initially diagnosed as choroidal hemangioma.Case reportA 42-year-old woman presented to Kurume University Hospital in November 1992 with an orange-yellow tumor nasal to the optic disc in the left fundus. The tumor was 9.0 × 9.0 mm in diameter, 6.0 mm thick, and was characterized by high intensity on T1-weighted magnetic resonance imaging (MRI), low intensity on T2-weighted MRI, and enhancement on gadolinium MRI. Fluorescein angiography revealed early hypofluorescence and late hyperfluorescence of the tumor and retinal feeder vessels. By April 1996, exudate had developed around the tumor margins. The patient was treated with external beam radiation therapy (20 Gy) in July 1996, but the tumor did not diminish in size. Subsequently, she developed extensive loss of vision due to total retinal detachment. Accordingly, her left eye was enucleated in June 2005 because of severe ocular pain due to absolute glaucoma. Histopathological examination indicated that the tumor was contiguous with the normal surrounding RPE and was composed of cords and tubules of mostly non-pigmented spindle-shaped cells with round to oval nuclei and a small amount of cytoplasm containing melanin granules. The tumor cells were immunoreactive for vimentin, S-100 protein, and cytokeratin 18. The final diagnosis was adenoma of the RPE.ConclusionAdenoma of the retinal pigment epithelium may be associated with incompetent vessels leading to serous retinal detachment and extensive visual loss, and may exhibit clinical characteristics similar to choroidal hemangioma.

Macular Pucker in Association with RPE Adenoma: A Report of a Case and Review of the Literature

“…Immunohistochemistry may reveal these tumors to be positive for epithelial membrane antigen, S-100, neurone-specific enolase, synaptophysin and vimentin, and negative for HMB45 and cytokeratin-7. 4 In the review by Shields et al, two of 13 RPE adenomas were found coincident with macular pucker. 3 Considering the RPE's ability to both migrate and produce collagen, and the significant amount of RPE produced by an adenoma, it is likely that some of these RPE cells may migrate transvitreally to the macula where collagen deposition may contribute to membrane formation.…”

“…RPE adenomas are more likely to be associated with vitreous cell, which may increase over time as pigment from the lesion sheds. 3,4 They have been reported to be associated with yellow exudates and may appear to have a feeding retinal artery and draining retinal vein. 5,6 Angiographic features include early hypofluorescence and intense late hyperfluorescence.…”

“…5,6 Angiographic features include early hypofluorescence and intense late hyperfluorescence. [3][4][5] Ultrasonography demonstrates high internal reflectivities on A-scan. 3,4 Fine-needle aspiration biopsy has been reported to show plump, pigmented round cells with large melanosomes.…”

“…[3][4][5] Ultrasonography demonstrates high internal reflectivities on A-scan. 3,4 Fine-needle aspiration biopsy has been reported to show plump, pigmented round cells with large melanosomes. In this case, microscopic examination revealed cords of cell with round or oval nuclei and rare mitotic figures.…”

Treatment Outcome of Acquired Retinal Pigment Epithelial Tumors with Rhuthenium-106 Plaque Radiotherapy

“…Therefore, some ocular oncologists prefer enucleation to achieve definite diagnosis and treatment. 4 5 6 7 8 However, limited trials of other modalities such as local tumor resection, 9 10 11 laser photocoagulation, 12 cryotherapy, 3 12 13 radiation, 3 11 and a combination of them have also been reported to preserve the globe and a trend away from enucleation as much as possible. In this study, we report our experience of two patients with acquired RPE tumors who were managed by rhuthenium-106 plaque radiotherapy as a globe preservation approach.…”