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 some 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
Tumor seeding into the chamber angle
Pigment release into the aqueous, the surface of the iris, and the chamber angle

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, secondary glaucoma as a result of intraocular tumors is a rare event.

Mortality/Morbidity

Early detection of malignant melanoma of the choroid is critical because a delayed diagnosis may lead to mortality from metastatic disease.

Prognosis

With early detection, the outlook is improved.

Patient Education

Follow-up care is important.

For patient education resources, see the Glaucoma Center, as well as Glaucoma Overview, Glaucoma FAQs, and Understanding Glaucoma Medications.

Clinical Presentation

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Author

Andrew A Dahl, MD, FACS Assistant Professor of Surgery (Ophthalmology), New York College of Medicine (NYCOM); Director of Residency Ophthalmology Training, The Institute for Family Health and Mid-Hudson Family Practice Residency Program; Staff Ophthalmologist, Telluride Medical Center

Andrew A Dahl, MD, FACS is a member of the following medical societies: American Academy of Ophthalmology, American College of Surgeons, American Intraocular Lens Society, American Medical Association, American Society of Cataract and Refractive Surgery, Contact Lens Association of Ophthalmologists, Medical Society of the State of New York, New York State Ophthalmological Society, Outpatient Ophthalmic Surgery Society

Disclosure: Nothing to disclose.

Coauthor(s)

Shehab A Ebrahim, MD Assistant Professor, Department of Ophthalmology, Tulane University; Vitreoretinal Surgeon, The Retina Institute, LLC

Shehab A Ebrahim, MD is a member of the following medical societies: American Academy of Ophthalmology, American Medical Association, Association for Research in Vision and Ophthalmology, American Society of Retina Specialists

Disclosure: Nothing to disclose.

Specialty Editor Board

Simon K Law, MD, PharmD Clinical Professor of Health Sciences, Department of Ophthalmology, Jules Stein Eye Institute, University of California, Los Angeles, David Geffen School of Medicine

Simon K Law, MD, PharmD is a member of the following medical societies: American Academy of Ophthalmology, Association for Research in Vision and Ophthalmology, American Glaucoma Society

Disclosure: Nothing to disclose.

Martin B Wax, MD Professor, Department of Ophthalmology, University of Texas Southwestern Medical School; Vice President, Research and Development, Head, Ophthalmology Discovery Research and Preclinical Sciences, Alcon Laboratories, Inc

Martin B Wax, MD is a member of the following medical societies: American Academy of Ophthalmology, American Glaucoma Society, Society for Neuroscience

Disclosure: Nothing to disclose.

Chief Editor

Hampton Roy, Sr, MD † Associate Clinical Professor, Department of Ophthalmology, University of Arkansas for Medical Sciences

Hampton Roy, Sr, MD is a member of the following medical societies: American Academy of Ophthalmology, American College of Surgeons, Pan-American Association of Ophthalmology

Disclosure: Nothing to disclose.

Additional Contributors

Andrew I Rabinowitz, MD Director of Glaucoma Service, Barnet Dulaney Perkins Eye Center

Andrew I Rabinowitz, MD is a member of the following medical societies: Aerospace Medical Association, American Academy of Ophthalmology, American Society for Laser Medicine and Surgery, American Academy of Ophthalmology, American Medical Association

Disclosure: Nothing to disclose.