Abstract
Background
Ciliary body tumors can remain undetected and achieve large dimensions. Pigmented ciliary body tumors include: melanoma, leiomyoma and melanocytoma, however correct diagnosis may require tissue diagnosis with immunohistochemical stains.
Case presentation
Two men presented with identical ciliochoroidal tumors. Both had darkly pigmented dome-shaped anterior uveal masses, exudative retinal detachments and transillumination shadowing. Ocular PET-CT imaging revealed that both were metabolically active consistent with a diagnosis of cancer. However, immunohistochemical examination revealed one a leiomyoma and the other melanoma.
Conclusion
Uveal leiomyoma can be an indistinguishable doppelgänger to ciliochoroidal melanoma, where the diagnosis can only be established by immunohistopathology.
Introduction
Uveal leiomyoma is a rare benign tumor of neural crest cell origin which can be clinically indistinguishable from a uveal melanoma. A recent study reviewed 80 published cases of ocular leiomyoma from 1899 to 2019 and found that pigmented cilio-choroidal tumors clinically diagnosed as large uveal melanoma were most commonly treated by enucleation. However, despite confounding clinical similarities, these 2 tumors have very different prognoses. Though both can cause secondary retinal detachment, macular edema and even extraocular extension, leiomyomas are benign tumors which do not metastasize. Herein, we present 2 anterior uveal tumors which are clinically identical. These doppelgänger ciliary body lesions highlight that pre-enucleation differentiation may not be possible clinically, the need for a high index of suspicion, and use of histopathology with immunohistochemical analysis to achieve the correct diagnosis.
Case presentation
Two patients presented to us with ciliochoroidal tumors with nearly identical clinical presentation. The first patient was a 30-year-old male referred for evaluation of a ciliary body tumor in his right eye. The second patient was a 45-year-old male with a 3-month history of blurred vision in the left eye and a similar ciliary body tumor with nearly equivalent epidemiologic and clinical features ( Table 1 ).
| Feature | Patient 1 | Patient 2 |
|---|---|---|
| Age | 30 years | 45 years |
| Gender | Male | Male |
| Race | Middle-Eastern | Hispanic |
| Chief Complaint | OD metamorphopsia | OS blurred vision |
| Best Corrected Visual Acuity | 20/20 OU | 20/20 OU |
| Anterior Segment examination |
|
|
| Intraocular pressure | 16 mm Hg | 14 mm Hg |
| Gonioscopy |
|
|
| Dilated fundus examination |
|
|
| Transscleral ophthalmic transillumination | Blocked light transmission | Blocked light transmission |
| High frequency ultrasound imaging (35 MHz) |
|
|
| Low frequency B-scan ultrasound (12 MHz) |
|
|
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