Ординатура / Офтальмология / Английские материалы / Tumors of the Eye and Ocular Adnexa_Char_2001

Figure 6–28. Clinical photograph of an extramacular disciform lesion simulating a uveal melanoma. Hemorrhage is very uncommon with a choroidal melanoma, unless it is thick enough to have broken through Bruch’s membrane.

Figure 6–29. Example of an extramacular disciform lesion with exudate and hemorrhage.

sonographic pattern of subretinal hemorrhage on both A-scan and B-scan can simulate a melanoma; however, often the acoustic quiet zone is not as homogeneous on B-scan, and on A-scan, there are no vascular pulsations and higher internal reflectivity (Figures 6–31A and B). The fluorescein angiogram in these patients with AMD and extramacular disciform (EMD) lesions is sometimes useful; there is no intrinsic tumor vasculature, and the underlying choroidal vasculature is obscured by hemorrhage (Figure 6–32). In patients referred to an oncology unit because of a diffuse vitreous hemorrhage, it is more likely caused by an extraocular disciform

lesion than by a uveal melanoma, particularly if the lesion is < 5 mm in elevation. While MRI may be useful, given that on acute hemorrhage has a pattern different from that of a melanoma, we have observed a number of errors where MRI scans had been used to attempt to establish the diagnosis. The patient shown in Figure 6–33 had an MRI pattern consistent with a melanoma. As seen in Figure 6–34, a serial observation demonstrated shrinkage of this EMD lesion. These lesions can also sometimes simulate an RPE rip.62 Rarely, we have observed these lesions to enlarge transiently. Figures 6–35A and B demonstrate such a case in which the patient was followed

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Figure 6–32. A fluorescein angiogram of a subretinal hemorrhage showing no intrinsic vasculature and blockage.

Figure 6–33. A clinical photograph of extramacular disciform with MRI features of uveal melanoma.

Figure 6–34. Serial follow-up shows involution of the lesion shown in Figure 6–33.

up for a presumed extramacullar disciform. When it enlarged > 2 mm in thickness, an FNAB was performed, which demonstrated hemosiderin-laden macrophages (Figure 6–36).

MISCELLANEOUS SIMULATING LESIONS

As discussed above, rhegmatogenous retinal detachment, RPE hyperplasia, and RPE hypertrophy can all simulate a melanoma; however, these conditions can almost always be differentiated on the basis of indirect ophthalmoscopy findings alone.

A melanocytoma not involving the optic disc can be difficult to diagnose; a uniform jet black color is more consistent with a diagnosis of melanocytoma than one of uveal melanoma.62,63 Rarely, melanocytomas present in children and diffusely involve the

A

B

Figure 6–35. A, Extramacular disciform lesion prior to growth. B, Same patient 6 months later, demonstrating a 2-mm enlargement of the lesion without any obvious hemorrhage.

Figure 6–36. Fine-needle biopsy of the lesion shown in Figure 6–35 with hemosiderin-laden macrophages. This lesion shrank over the next year.

choroid.63 We have monitored the lesion shown in Figure 6–37, a presumed peripheral uveal melanocytoma, for 20 years and observed no change. Melanocytomas can undergo necrosis, and in one report, 10 of 23 had that feature. Figure 6–38 shows an FNABcon- firmed melanocytoma approximately 8 mm thick. Six years after biopsy, the lesion remained stable, and vision was still 20/20. As discussed previously, melanocytoma can perfectly simulate the MRI or ultrasound pattern of a melanoma, as was noted in this case. Occasionally, they can also undergo malignant degeneration.64,65

A choroidal osteoma can appear similar to a metastatic tumor, sclerochoroidal calcification, or an amelanotic melanoma on ophthalmoscopic examina- tion.66–71 Clinically, choroidal osteomas are amelanotic and relatively flat with an irregular surface and may grow. A typical lesion is shown in Figure 6–39A. On fluorescein angiography, there is often an area of capillary tufting circumferentially (Figure 6–39B). Intralesional calcium is visible by ultrasonography or computed tomography (CT) (Figures 6–39C and D). While intralesional calcium is classic for an osteoma, we as well as others have seen that only rarely in choroidal melanomas.72 Usually, osteomas are more common in women in their 20s and 30s, and approximately 75 percent are unilateral.73

Approximately 70 choroidal osteomas have been reported. They usually occur near the optic nerve. Rarely, they have been reported to have developed in later life; two siblings were noted to have normal fundi at an earlier age and then developed this process.74 Occasionally, patients develop these lesions in an area of previous uveitis, and one-quarter of cases have been bilateral. In young children, these lesions can occasionally be diagnosed when there is rapid enlargement.75 In other familial reports, bilateral disease has been documented.76 While initially most patients have had good vision, almost one-half eventually develop poor vision due to retinal atrophy

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or subretinal neovascularization. In a series from the Bascom Palmer Institute, with a mean follow-up of 10 years, growth was observed in 9 of the 36 patients studied and visual loss to ≤ 20/200 was noted in 58 percent of the cases, mainly from choroidal vascularization.77

Choroidal detachments, especially those after cataract surgery, can also be mistaken for melanoma.78 While these usually occur after prolonged hypotony and involve a large area of the

choroid, they may be focal (Figures 6–40A and B) or diffuse (Figure 6–41). Most commonly, they dissipate within 6 weeks; on fluorescein angiography, the lesion has no intrinsic vascular pattern (Figure 6–42). Ultrasonography is not diagnostic; the A- or B-scan pattern can simulate melanoma (Figures 6–43A and B) in these focal lesions. Rarely, these lesions can be as thick as 5.5 mm.79

Posterior scleritis can simulate an amelanotic melanoma. Most patients are female, and this entity

often occurs in association with rheumatoid disease.80 Patients often present with a deep boring pain, especially during ocular versions. The ultrasonographic pattern (Figure 6–44), often with fluid in Tenon’s space, is typical; the CT pattern, as shown in Figure 6–45, is also characteristic. Clinically, there may be an associated exudative detachment over an amelanotic lesion, with associated choroidal folds. Occasionally, anterior extension of the scleritis is noted. Rarely, a metastatic focus can simulate this entity, as in the testicular uveal metastasis shown in Figure 6–46. Rarely, a plaque-like melanoma can simulate scleritis and even partially respond to corticosteroids.81,82

There are a myriad of other lesions that can simulate uveal melanoma.83 As an example, in the patient shown in Figures 6–47A and B, there was a longstanding rheumatoid arthritis and probably an old inactive scleritis. Alternatively, this lesion could have been a herniation of the vortex vein wall, but the author thinks that is less likely. On the patient looking up, a small tumor was noted (see Figure 6–47A). When the patient looked down, the tumor disappeared.

Rarely, lymphoid lesions can simulate uveal melanoma. Many of these tumors will have a classic fleshy pink subconjunctival change in association with diffuse choroidal thickening. Often, on ultrasonography, these lesions have vascularity noted.83–85

tunately, many of these unusual simulating lesions cannot be correctly diagnosed with noninvasive techniques. As discussed in a later chapter, FNAB may decrease enucleation of these very atypical simulating lesions. There are insufficient data to determine the diagnostic accuracy and sensitivity of that technique with atypical pseudomelanomas. In one report, however, FNAB data altered the therapeutic plan in 48 percent of the patients who had this procedure.93 In our experience, as discussed in Chapter 8, it has altered the management in about 10 percent of cases.

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