Background
Intraocular tumors are rare causes of glaucoma. In cases of unexplained glaucoma, the possibility of an ocular tumor must be considered because of the dire consequences of a missed diagnosis. The epidemiology, prognosis, and mechanism of action depend on the specific tumor type. The tumor may not be directly visible in many instances, and different methods of indirect visualization may need to be used.
Pathophysiology
There are a number of different mechanisms by which ocular tumors can cause glaucoma: (1) direct invasion, (2) pigment dispersion, (3) melanophagic, (4) hemolytic, (5) uveitic, (6) secondary angle closure, (7) iris neovascularization, (8) choroidal detachment, (9) suprachoroidal hemorrhage, and (10) anterior displacement of lens-iris diaphragm.
Mechanisms of elevated intraocular pressure in intraocular malignant melanoma, which is the most common intraocular tumor causing secondary glaucoma, include the following:
Melanoma arising within the iris
- Direct growth of the tumor into the angle
- Tumor seeding into the angle
- Pigment release into the anterior chamber and into the angle
Melanoma arising within the ciliary body
- Pigment dispersion
- Direct invasion
Melanoma arising within the choroid
- Neovascularization of the angle
- Anterior displacement of lens-iris diaphragm with angle closure
- Hemorrhage with volume effect or angle closure
- Spontaneous necrosis causing uveitis and secondary glaucoma
Epidemiology
Frequency
United States
Although individual rates vary, Shields et al showed a 5% incidence of increased intraocular pressure due to intraocular tumors in a series of 2597 patients with ocular tumors.[1] This is in contrast to 50% incidence of increased intraocular pressure in eyes enucleated for tumors. Incidence of increased intraocular pressure also is dependent on location. Reports indicate a 17% incidence of glaucoma in ciliary body melanoma, 7% in iris melanoma, and 2% in choroidal melanoma. Given the relative infrequency of intraocular tumors, this condition is a rare event.
Mortality/Morbidity
Early detection of this rare tumor is important because it may increase mortality.
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