Background Information
Choroidal melanoma is the most common intraocular tumor. It is a form of uveal melanoma, representing about 80% of uveal melanoma cases. These tumors grow within the choroid, the vascular layer directly beneath the retina, which is responsible for providing oxygen and nutrients to the retinal cells. Choroidal melanoma is usually diagnosed at an average age of about 60 years. It presents most commonly in Caucasian patients and those with lighter eye color. Patients may experience some visual symptoms depending on the size, location of the tumor within the eye, and the amount of subretinal fluid present.
Symptoms
Patients with choroidal melanoma often do not experience symptoms at all and the tumor is instead found during a regular eye examination. When symptoms are present, they most commonly include photopsia (perception of flashing lights) or metamorphosia (visual distortion).
Signs
Fundoscopy, the direct visual examination of the lesion performed by your Ophthalmologist, is the primary test utilized in the diagnosis of uveal melanoma. Ultrasound, which uses sound waves for examination of the eye interior, uses the internal reflectivity of tumor cells to allow for evaluation of tumor size, shape, and progression. Most choroidal melanomas are dome-shaped; however, if Bruch’s membrane (the innermost choroidal membrane) is ruptured , the tumor will have a mushroom/collar-button shape. Pigmentation of choroidal melanoma can vary, ranging from dark brown to non-pigmented.
Treatments
Decision for treatment of choroidal melanoma is made on an individualized basis. When deemed necessary, treatment with radiation therapy for small to medium sized tumors or eye removal (enucleation) in cases of large tumors. Plaque radiation therapy (brachytherapy) is the most common treatment for uveal melanoma and has been shown to be just as effective as enucleation on overall survival.
What to Expect
Choroidal melanoma may result in decreased visual acuity, with the potential for partial or total vision loss in the affected eye. Choroidal melanoma has the ability in some patients to spread to other parts of the body and is therefore classified as a malignant tumor. Prognosis of choroidal melanoma depends on tumor size and whether or not development of tumor metastasis (spreading to other parts of the body) occurs. These factors should be discussed with an individual’s Ophthalmologist.
Background Information
Ciliary body melanoma can occur in either a circumscribed or annular (ring) form. The ciliary body, which is located directly behind the iris and in front of the choroid, is responsible for producing the aqueous fluid that fills the anterior region of the eye. In addition, contraction/relaxation of the ciliary body allows for accommodation, the process by which the shape of the lens changes to allow the eye to focus on objects at varying distances. Ciliary body melanoma originates in melanocytes (melanin-producing cells), which are located in the ciliary body.
Symptoms
Patients with iris melanoma are usually asymptomatic. When symptoms are present, they may include irregular astigmatism, secondary glaucoma, cataract, or tumor in the line of vision (visual axis).
Signs
Use of techniques such as gonioscopy, slit lamp examination, ultrasound biomicroscopy, and transillumination help eye the Ophthalmologist diagnose your tumor.
Treatments
When deemed necessary, small tumors may be resected or treated with radiation therapy. Larger tumors may require enucleation (removal of the eye).
What to Expect
Ciliary body melanoma may result in a decrease in visual acuity. Ciliary body melanoma has the ability in some patients to spread to other parts of the body and is therefore classified as a malignant tumor. Prognosis of ciliary body melanoma depends on whether or not development of tumor metastasis (spreading to other parts of the body) occurs and should be discussed with an individual’s Ophthalmologist.
Background Information
Iris melanoma can occur in either a circumscribed or diffuse form. Both forms affect the iris, which is the colored part of the eye.
Circumscribed iris melanoma tumors are nodular in shape and may contain varying degrees of pigmentation. In contrast, diffuse iris melanoma only show a diffuse thickening of the entire iris.
Symptoms
Patients with iris melanoma are usually asymptomatic. Diagnosis of tumor presence usually occurs when the tumor is noticed by the patient, their family members, or an eye care professional during a routine examination. Since many people tend to have freckles on their irises, the individual should consult with an Ophthalmologst to diagnose these lesions and arrange appropriate follow-up or treatment.
Signs
Ultrasound biomicroscopy, which uses sound waves for examination of the eye interior, can be used to examine the depth and extension of the iris melanoma. In addition, ultrasound examination can provide information regarding whether the tumor is cystic or solid. Several characteristics exist that may suggest a cancerous nature of the tumor. These characteristics include secondary glaucoma, cataract development, formation of blood vessels with the tumor (intrinsic vascularity), and pupil deformation (ectropion uveae).
Treatments
When necessary, iris melanomas can be completely removed with surgical excision leaving the vision in that eye functioning at a high level. Other times, depending on the tumor characteristics, iris melanomas may be treated with plaque radiotherapy which also carrying excellent visual prognosis. Large iris melanomas, however, often require removal of the eye (enucleation).
What to Expect
Fortunately, iris melanomas rarely spread to other parts of the body
and thus have a generally good prognosis in terms of survival and vision.
Background Information
Circumscribed choroidal hemangioma is most commonly diagnosed between the ages of 20-50. These lesions, which are made up of blood vessels, grow within the choroid, the vascular layer directly beneath the retina. Choroidal hemangiomas may cause visual disturbances due to fluid leakage. Fortunately, this vascular anomaly is benign, and therefore does not metastasize (spread to other parts of the body).
Symptoms
The most common symptom of circumscribed choroidal hemangioma is visual disturbance. These disturbances can include blurred vision, metamorphosia (visual distortion), flashing lights, or hyperopia (far-sightedness). However, choroidal hemangiomas often do not cause symptoms and are instead found during regular eye examinations. These lesions are typically masses that are reddish to orange in color and blend into the surrounding choroid at the margins.
Signs
Fundoscopy (direct visualization of the back of the eye) is the primary method an ophthalmologist diagnoses these lesions. Ultrasound and angiography using fluorescein or indocyanine green (ICG), which are synthetic organic dyes, are also commonly used methods for diagnosing circumscribed choroidal hemangiomas. During an ultrasound examination, sound waves are used for examination of the eye interior. The blood vessels making up the choroidal hemangioma reflect sound waves intensely, resulting in the high internal reflectivity that is characteristic of these tumors. Fluorescein or ICG angiography allows for visualization of the ocular blood vessels through injection of the organic dye into the patient’s arm. From there, the dye travels to blood vessels in the eye, where it is photographed with special filters to visualize and characterize the blood vessel patterns that help distinguish hemangiomas from other masses.
Treatments
Decision for treatment of circumscribed choroidal hemangioma is made on an individualized basis and depends on the extent of symptoms, vision loss, and the potential for recovery of vision. Asymptomatic cases in which the tumor is not located in the macula and does not have surrounding subretinal fluid can be kept under observation without need for further treatment. In addition, cases with very limited potential for visual recovery may also be kept under observation. Cases for which treatment is deemed appropriate are commonly treated with one or more of the following options:
Photodynamic therapy. A safe and effective course of treatment, photodynamic therapy has minimal complications and offers site-specific destruction of choroidal tumors. It is a form of laser therapy that uses non-toxic light-sensitive compounds, which are injected into the patient’s vein. The hemangioma is then selectively exposed to light, activating the light-sensitive compound and causing closure of abnormal blood vessels within the tumor.
Intra-vitreal avastin. The injection of avastin into the eye to decrease the fluid leakage has been used. Early experiences are promising, but many Ophthalmologists feel that this treatment may not be long-lasting. Combined treatment with avastin as a first step, because of its quick action, and followed by photodynamic therapy is emerging as a common treatment algorithm for circumscribed choroidal hemangioma.
Laser photocoagulation. This course of treatment does not result in significant tumor regression but allows for a decrease in subretinal fluid build-up. Unfortunately, up to 40% of cases treated with laser photocoagulation required additional treatment due to the recurrence of subretinal fluid.
Radiation treatment. Several forms of radiotherapy for circumscribed choroidal hemangioma exist, including: external beam radiotherapy, stereotactic radiotherapy, plaque radiotherapy (brachytherapy), and proton beam radiotherapy. Radiation treatment has been reported to result in tumor regression, improvement of vision, and resolution of retinal detachment as a result of subretinal fluid presence.
Thermotherapy. Over 90% of cases treated with thermotherapy can be expected to result in complete or partial regression of the tumor with corresponding visual improvement.
What to Expect
The chronic presence of subretinal fluid and cystoid macular edema may result in compromise of visual acuity. In addition, side effects of treatments for circumscribed choroidal hemangioma may cause reductions in vision. It should be noted, however, that, with increasing usage of photodynamic therapy, achievement of better long-term results may be possible. In addition, it is important to emphasize that these tumors are benign and do not metastasize (spread to other parts of the body).
Choroidal Osteoma
Background Information
Also referred to as osseous choristoma, choroidal osteomas are bone-like tumors that grow within the choroid, the vascular layer beneath the retina. Most cases occur sporadically; however familial cases have also been reported. The mean age of diagnosis of choroidal osteoma is 21 years. Approximately 90% of affected individuals are women and the majority of cases are unilateral, affecting only one eye. Choroidal osteomas tend to result in focal depigmentation of the retinal pigment epithelium (RPE), which is the cell layer located directly in between the choroid and the retina. This depigmentation is the result of the tumor’s impedance of blood flow to the overlying retina as it causes narrowing and/or loss of choriocapillaries.
Symptoms
Although most patients with choroidal osteoma do not experience any symptoms, some common symptoms include visual field defects, metamorphosia (visual distortion), and loss of vision. The tumor, which is often well-defined and round/oval with wavy margins, is yellowish-white in color and sometimes contains clumps of brown/black pigment on its surface. Choroidal osteomas are usually flat, less than 2.5 mm in thickness, and often located close to the optic nerve head. While only about 40% of choroidal osteoma tumors grow, growth is typically rather slow and spontaneous reabsorption or decalcification may occur. The major complication associated with this type of tumor is choroidal neovascularization, the growth of abnormal small vascular networks in the choroid, and is usually kept under observation by an Ophthalmologist.
Signs
Fundoscopy (direct visualization of the back of the eye) is the primary method an ophthalmologist diagnoses these lesions. Angiography, which uses fluorescein or indocyanine green (ICG) synthetic organic dyes, allows for visualization of the ocular blood vessels through injection of these dyes into the patient’s hand or arm. From there, the dye travels to blood vessels in the eye, where it is used to visualize block perfusion patterns characteristic of choroidal osteoma.
Ultrasound, which uses sound waves for examination of the eye interior, is the most helpful method in diagnosis of choroidal osteoma and can allow for measurement of tumor thickness. Choroidal osteomas are characteristically highly reflective of sound waves. Ultrasound examination will also usually demonstrate orbital shadowing.
Computer tomography (CT) can be used to visualize calcification of the tumor, often demonstrating an opaque, plaque-like region in the choroid.
Treatments
Decision for treatment of circumscribed choroidal osteoma is made on an individualized basis. Asymptomatic cases with an absence of complications that may threaten vision can be kept under observation without need for further treatment. Choroidal neovascularization, when present, may be treated with laser photocoagulation, anti-angiogenic agents, or photodynamic therapy (PDT). Vision loss resulting from choroidal osteoma itself is not usually treatable; however, it is important to emphasize that this is a benign tumor.
What to Expect
Visual acuity can decrease as a result of choroidal neovascularization and/or retinal pigment epithelium (RPE) degeneration if the osteoma is in a critical area of the eye. The tumor itself, however, is benign and very slow growing, if it grows at all.
COMMON CONJUNCTIVAL TUMORS
Squamous Cell Carcinoma of the Conjunctiva
Background Information
Squamous cell carcinomas of the conjunctiva are the most common malignant conjunctival tumor in adults. The average age of diagnosis is about 60 years old, with slightly higher rates in men than women. The conjunctiva is the skin that covers the eye and the inside of the eyelid. Ultraviolet (UV) exposure is an established risk factor for these tumors.
Symptoms
Patients with squamous cell carcinomas of the conjunctiva often present with an irregular mass on the surface of their eye. These lesions can occur in several different forms including: lesions with a gelatinous appearance, white, flesh-coloured, or red patches. When on the inside of the eyelid they might not be easily visible. Squamous cell carcinomas of the conjunctiva can cause chronic irritation of the eye or may cause no symptoms.
Signs
An Ophthalmologist can differentiate many non-cancerous lesions that can occur on the conjunctiva. But because the presentation of SCC can vary significantly, an important diagnostic evaluation for squamous cell carcinoma of the conjunctiva is biopsy. This method involves removing part or all of the lesion and sending it to the pathology lab where they prepare, stain, and examine the lesion under a microscope.This allows for histopathologic examination of the suspected tumor and serves as a confirmation method for diagnosis. The most important assessment to be made with these types of lesions is whether the lesion is contained by the basement membrane (a thin membrane made of protein that separates the epithelium from underlying tissue). Invasion beneath the basement membrane defines these lesions as carcinoma.
Treatments
Decision for treatment of conjunctival squamous cell carcinoma is made on a case-by-case basis. The most common treatment is surgical excision. When deemed necessary, topical chemotherapy, radiation therapy, may be used for treatment of squamous cell carcinoma of the conjunctiva.
What to Expect
Prognosis of conjunctival squamous cell carcinoma depends on tumor size, thickness, and degree of infiltration. Usually, these lesions are limited to the surface of the eye and can be completely resected, but they can sometimes extend into the eye and orbit requiring more aggressive surgical interventions. SCC can spread to other parts of the body, including the lymphatic tissue.
Primary Acquired Melanosis
Background Information
Primary acquired melanosis refers to relatively flat conjunctival lesions that are not the result of generalized pigmentation or congenital conditions. The term ‘melanosis’ refers to the fact that the pigmentation observed in these lesions is a result of the production of melanin and not any other pigment. In general, primary acquired melanosis is more prevalent in individuals with fair complexions as compared to those with darker skin tones. Some studies suggest that sun exposure may play a role in the development of these lesions; however, this association is not yet clear.
Symptoms
Primary acquired melanosis often presents as a flat brown conjunctival lesion, with pigmentation varying from golden to dark brown. Although most cases are unilateral, presenting in only one eye, bilateral lesions may also occur, in which both of the patient’s eyes are affected. Areas of these lesions have been observed to be variable in coloration, lightening or darkening as part of a “waxing and waning” phenomenon. These lesions can be subdivided into two categories: primary acquired melanosis with and without atypia.
Signs
An important diagnostic evaluation for primary acquired melanosis is a biopsy. These biopsies typically involve removing the entire lesion. This method of evaluation allows for the lesion to be examined under the microscrope with special stains that will confirm a diagnosis and let us know if the lesion has any atypical cells within it.
Treatments
Decision for treatment of primary acquired melanosis is made on an individualized basis. Some common forms of treatment following biopsy of the lesion include further surgery, chemotherapy, or observation.
What to Expect
Recurrence of primary acquired melanosis following excision of the lesion is fairly rare in cases without atypia; however, recurrence is more common in cases with atypia, often recurring as malignant melanoma. Factors that may increase the chance of recurrence include incomplete excision and involvement of the cornea.
Conjunctival Melanoma
Background Information
Conjunctival melanoma is a fairly rare conjunctival tumor, most often diagnosed between the ages of 40 and 70. Similar to primary acquired melanosis, these lesions are usually pigmented. Conjunctival melanoma tends to be more common in individuals with fair complexions as compared to those with darker skin tones. In addition, these lesions can be subdivided into two categories: those that are associated with primary acquired melanoma with atypia and those that are not.
Symptoms
Conjunctival melanomas tend to affect one side, and may be slightly elevated and dark. These tumors are typically pigmented but can also occur in an amelanotic form (without melanin/pigmentation). Conjunctival melanoma lesions are usually nodular and can occur in any region of the conjunctiva.
Signs
An important diagnostic evaluation for conjunctival melanoma is for an excisional biopsy, serving as a confirmation method for diagnosis.
Treatments
Decision for treatment of conjunctival melanoma is made on an individualized basis. When deemed necessary, the primary method of treatment for these tumors is the complete excision of the lesion. In some cases, supplemental cryotherapy and chemotherapy may be used in conjunction with surgical excision of the tumor. Radiation (utilizing a brachytherapy technique) post surgical excision is utilized in select cases.
What to Expect
Prognosis of conjunctival melanoma depends on tumor size, thickness, and degree of infiltration. Spreading of conjunctival melanoma to the eyeball or orbit (eye socket) is very rare; however, metastasis (spread of the tumor to other parts of the body) may occur. When it does occur, tumor metastasis most often affects the regional lymph nodes.