Optic Nerve Melanocytoma

By Paul T. Finger, MD

Description

Note the dark tumor located next to and within the optic nerve. The edges have a feathered appearance (arrow).
Note the dark tumor located next to and within the optic nerve. The edges have a feathered appearance (arrow).

Optic nerve melanocytoma is typically a benign tumor made up of melanocytes and melanin. They can grow, but rarely transform into a malignancy. However, local growth can harm adjacent tissues.

Symptoms

Optic nerve melanocytoma does not usually produce symptoms or grow. If they slowly grow, optic nerve melanocytoma can produce afferent pupillary defects (30%), subretinal fluid (10%), and an enlarged blind spot (75%).

For example, if the tumor is next to the optic nerve, growth can compress the nerve and cause loss of vision (e.g. nerve fiber layer defects). Growth can also cause compressive vascular problems like central retinal vein occlusion. Lastly, growth also causes the tumor to exceed its blood supply. In these cases, necrotic areas form inside the tumor. Necrosis can (in turn) cause intraocular and rarely orbital inflammation.

Diagnosis

Most optic nerve melanocytomas are small, black, and do not grow. A medium-sized juxtapapillary melanocytoma
Most optic nerve melanocytomas are small, black, and do not grow.
A medium-sized juxtapapillary melanocytoma
A small juxtapapillary melanocytoma (note the small feathered edge).
A small juxtapapillary melanocytoma (note the small feathered edge).

Unlike choroidal melanoma, optic nerve melanocytomas are black, commonly extend onto the surface of the optic nerve and invade the nerve fiber layer (feathered edge).

Treatments

Patients with optic nerve melanocytoma should have a visual field test, as well as a photograph of the optic nerve and tumor. These examinations can serve as a baseline for future comparison. Patients with optic nerve melanocytoma should be periodically examined for evidence of growth, loss of visual field and optic nerve compression.

Unfortunately, there are no current treatments to prevent or stop optic nerve melanocytoma growth. Patients are examined every 6 to 12 months with dilated ophthalmoscopy and visual field examinations as to counsel patients about the risk of vision loss, treat compression-related vasculopathy and monitor for the rare occurrence of malignant transformation.

Additional info

Though rare, tumor growth can lead to radiation or enucleation (removal of the eye). 

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Iris Melanocytoma

By Paul T. Finger, MD

Description

Melanocytoma is a form of nevus that can occur in and behind the iris in the ciliary body. In the image seen below, this melanocytoma has a cobblestone textured surface and feathered margins. Interestingly, it is also causing a small amount of pupillary distortion (correctopia). Bits of iris melanocytoma can break off and settle in the inferior angle. These sedimentary melanocytoma cells can clog the natural drain of the eye (trabecular meshwork), causing increased eye pressure (glaucoma). Melanocytoma tumors can grow, malignant transformation is rare and metastasis is reportable.

Symptoms

Most patients with iris melanocytoma can see a dark spot on their iris, and have no other symptoms. Patients can have pigment dispersion, secondary glaucoma and intraocular inflammation (iritis). Secondary glaucoma can either be asymptomatic, cause one-sided (ipsilateral) headaches and cloudy vision (with halos around lights).

Diagnosis

Iris melanocytoma can be diagnosed by clinical examination. The tumor tends to be dark brown to black and the edges feather-shaped. The surface can be cobblestone (bumpy) appearance or smooth.

High-frequency ultrasound is particularly helpful for the diagnosis of iris melanocytoma. This technique is used to evaluate the depth of penetration into the iris and surrounding tissues. It can also be used to monitor for growth. These tumors can extend through the iris and into the ciliary body. Unlike low reflective iris melanomas, they tend to appear bright (highly reflective).

Iris Melanocytoma Long
Longitudinally oriented 20 MHz high-frequency ultrasound demonstrates the club-shaped melanocytoma
Iris Melanocytoma (Transverse)
Transverse oriented 20 MHz high-frequency ultrasound demonstrates its cobblestone-shaped surface.

Treatments

Iris melanocytoma does not usually require treatment. High-quality slit-lamp and gonioscopic photographs should be taken to record the appearance and surface characteristics of the tumor. High-frequency ultrasound should be performed to evaluate its thickness and extension into surrounding structures. These evaluations will be used to monitor for growth. Careful attention should be made to measuring intraocular pressure. Secondary glaucoma can occur due to pigment that clogs the natural drain of the eye (trabecular meshwork). Patients should keep a copy of these baseline tests in case they move to another area or their doctors change.

Should an iris melanocytoma be found to grow, an iris biopsy can be performed to determine if it is benign or malignant. When possible, rapidly growing and malignant iris melanocytomas whould be surgically removed. If resection is not possible, they can be treated with relatively high-dose plaque radiation therapy.

Additional info

Secondary melanocytomalytic glaucoma can be treated like other glaucomas. It is reasonable to consult with a glaucoma specialist. Strenuous exercise may “shake-up” the intraocular pigment and cause a temporarily increased eye pressure. When this happens, patients may notice headaches or cloudy vision after exercise.

If glaucoma surgery is recommended (e.g. trabeculectomy, stent, valve), a tumor biopsy can ensure that the tumor is not a melanoma.

(See Innovations Section – Finger Iridectomy Technique).

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