This article is more than 5 years old. Information may no longer be current.
Suspicious lesion found in right eye of elderly woman
The mass protruded from behind the iris and extended into the pupillary axis.
Click Here to Manage Email Alerts
An 80-year-old white woman presented to her ophthalmologist for an annual checkup when a suspicious lesion was noted in the right eye. The lesion protruded from behind the iris but appeared to be anterior to the lens. The patient was visually asymptomatic at the time, but a decision was made to refer the patient to New England Eye Center for further evaluation.
The patient’s medical history was significant for hypertension, which was being treated with nifedipine and atenolol. Her ocular history was significant for cataract extraction with posterior chamber IOL placement in both eyes. She was not allergic to any medications and denied any tobacco or alcohol use. Her family history was unremarkable for any ocular or cancerous pathology.
Examination
| Shazia Ahmed | My Hanh T. Nguyen |
On examination, the patient had a best corrected visual acuity of 20/60 in the right eye and 20/30 in the left eye. The right pupil appeared irregular in shape while the left pupil was brisk and reactive to light. Applanation tonometry revealed an IOP of 13 mm Hg in the right eye and 18 mm Hg in the left eye. Anterior segment slit-lamp examination revealed white, quiet eyes. The corneas appeared clear, and the anterior chamber was deep in both eyes. In the right eye, focal elevation of the iris was noted superotemporally with a brownish vascular mass extending into the pupillary axis. The approximate dimensions of this mass by slit-lamp measurement were 5 mm by 3.7 mm (Figure 1). Ultrasound biomicroscopy was performed, showing a large ciliary body mass extending from 11 to 12 o’clock with localized angle occlusion and a centralized fluid pocket (Figure 2). A B-scan demonstrated a suspicious elevation into the vitreous cavity at the 11 o’clock location with a height of 7.4 mm and a diameter of 9.6 mm (Figure 3).
| External photograph of the right eye showing brownish mass elevating the iris superotemporally and extending into the pupillary axis. Ultrasound biomicroscopy of a large ciliary body mass extending from 11 to 12 o’clock with localized angle occlusion and a centralized fluid pocket. B-scan of lesion extending into the vitreous cavity at the 11 o’clock location with a height of 7.4 mm and a diameter of 9.6 mm. Images: Ahmed S, Reichel E |
What is your diagnosis?
Suspicious lesion
The differential diagnosis is extensive and can be divided into cystic masses vs. solid tumors. Given the presence of a centralized fluid pocket on ultrasound biomicroscopy, iris pigment epithelial cysts and ciliary body cysts are part of the differential. Iris pigment epithelial cysts tend to be thin-walled with a central area of lucency that represents the majority of the lesion. In addition, the average diameter of the cyst is 2.4 mm, considerably smaller in size than our lesion. Solid tumors to consider include ciliary body melanoma, ciliary body melanocytoma, adenoma of ciliary pigment epithelium, schwannoma, leiomyoma and medulloepithelioma. Leiomyoma tends to be a nonpigmented tumor found mostly in young women. Medulloepithelioma is likewise nonpigmented but tends to occur mostly in children.
At the time of presentation, the patient was given a diagnosis of iris pigment epithelial cyst. The decision was made to closely follow the patient. Three months later she returned with the complaint of decreased vision in the right eye. On exam the lesion had grown considerably in size, filling 40% of the anterior chamber and extending further into the visual axis (Figure 4). The posterior chamber IOL was subluxated inferonasally. A prominent episcleral sentinel vessel was also noted. The patient was referred to an ocular oncologist for further evaluation. On repeat measurement by ultrasonography, the mass was found to be 16 mm in diameter and 14 mm in thickness. Ultrasound biomicroscopy revealed a mass with multiple echolucent cavities. B-scan disclosed a mushroom-shaped mass arising from the ciliary body with a large cavity at the base. A diagnosis of cavitary ciliary body melanoma was made, and the patient underwent subsequent enucleation. Histopathology confirmed this diagnosis. Figures 5A, 5B and 5C demonstrate on a gross and microscopic level a large mass extending from the ciliary body and eroding through the iris. Multiple cavitations existed within the mass. It was composed of spindle and epithelioid melanoma cells, which confirmed the diagnosis. No extrascleral extension of the tumor was found.
|
|
|
|
|
|
Discussion
Cavitary ciliary body melanoma is an extremely rare entity with only 26 reported cases in the literature. It is often misdiagnosed as a benign cyst given the presence of cavitation. In many cases these tumors are an incidental finding, but they can also lead to decreased vision secondary to the mass inducing lens subluxation, cataract formation, glaucoma or retinal detachment. Clinically, the tumor may present as a brown mass of variable size that is diffuse or nodular. Despite the presence of cavitation, these tumors block transmission of light on scleral transillumination. The presence of an episcleral sentinel vessel should herald a diagnosis of uveal melanoma. Often these tumors have their own intrinsic vasculature. IOP can be unilaterally low in these eyes.
On ultrasound, one or more cystic cavities may exist. These cavities possess thick walls of medium reflectivity. The wall thickness of these lesions tends to be 0.4 mm or greater whereas a true pigment epithelial cyst has a wall thickness of 0.2 mm or less. On average the cavities occupy 55% of the mass thickness. The composition of these cavities range from being completely empty to including serous fluid, erythrocytes or pigment-laden macrophages. No signs of necrosis or the presence of epithelial or endothelial cells were found in a case series by Lois and colleagues.
Factors to consider that affect patient prognosis include tumor thickness, anterior location of tumor margin, cell type and the presence of metastasis. Prognosis of melanoma based on cell type reveals that the epithelioid variety is associated with a higher rate of morbidity when compared with the spindle type. Metastasis may occur through one of two routes: extraocular extension vs. hematogenous dissemination. Sites of tumor metastasis include liver, lung, bone, skin and the central nervous system. Ocular workup includes a thorough eye exam, A scan/B scan, ultrasound biomicroscopy and fine needle aspiration. A systemic workup should include checking liver enzyme levels, abdominal imaging and a chest X-ray.
Treatment is based on tumor size and characteristics. Tumors less than 3 mm in thickness may be safely observed. Exceptions to this rule are rapidly growing tumors, vascular tumors and tumors eroding through the iris. Resection is efficacious when less than 4 clock hours of the eye are involved. Radiation treatment is effective for tumors involving 4 or more clock hours with choroidal involvement. A diameter of 13 mm with a thickness of 7 mm represents the average dimensions of a tumor undergoing plaque brachytherapy. Side effects of radiation treatment include cataract formation, neovascular glaucoma, radiation retinopathy, scleral necrosis and vitreous hemorrhage. Enucleation is the preferred option in cases in which tumor thickness is greater than 10 mm or base diameter is greater than 20 mm. Tumors involving 180° or more of the angle should always undergo enucleation.
In summary, this case presentation highlights the easy misinterpretation of a ciliary body melanoma for an iris pigment epithelial cyst. Clinicians should be aware that a cavitary lesion does not exclude melanoma from the differential diagnosis and further careful clinical evaluation is warranted.
For more information:
- Shazia Ahmed, MD, and Elias Reichel, MD, can be reached at New England Eye Center, Tufts University School of Medicine, 750 Washington St., Box 450, Boston, MA 02111; 617-636-4219; fax: 617-636-4866; Web site: www.neec.com.
- Edited by Shazia Ahmed, MD, and My Hanh T. Nguyen, MD. Drs. Ahmed and Nguyen can be reached at New England Eye Center, Tufts University School of Medicine, 750 Washington St., Box 450, Boston, MA 02111; 617-636-4219; fax: 617-636-4866; Web site: www.neec.com. Drs. Ahmed and Nguyen have no direct financial interest in the products mentioned in this article, nor are they paid consultants for any companies mentioned.
References:
- Char DH, Miller T, Crawford JB. Uveal tumour resection. Br J Ophthalmol. 2001;85(10):1213-1219.
- Conway RM, Chew T, et al. Ultrasound biomicroscopy: role in diagnosis and management of 130 consecutive patients evaluated for anterior segment tumours. Br J Ophthalmol. 2005;89(8):950-955.
- Criss JS, Shields CL, et al. Progressive enlargement of cavity within melanoma masquerading as iris cyst. Cornea. 2006;25(7):863-865.
- De Potter P, Shields CL, Shields JA. New treatment modalities for uveal melanoma. Curr Opin Ophthalmol. 1996;7(3):27-32.
- Finger PT. Plaque radiation therapy for malignant melanoma of the iris and ciliary body. Am J Ophthalmol. 2001;132(3):328-335.
- Gunduz K, Shields CL, et al. Plaque radiotherapy of uveal melanoma with predominant ciliary body involvement. Arch Ophthalmol. 1999;117(2):170-177.
- Lois N, Shields CL, et al. Cavitary melanoma of the ciliary body. A study of eight cases. Ophthalmology. 1998;105(6):1091-1098.