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OCULAR TUMORS

Intraocular tumors are evaluated with a variety of diagnostic imaging technologies to make a diagnosis and create a personalized clinical plan. Benign lesions such as choroidal nevi, hemangiomas, and osteomas are typically monitored but may be treated to improve vision. Malignancies such as uveal melanoma, or metastasis of cancer to the eye, are treated in coordination with oncology teams. Procedures offered may include vitrectomy, biopsy, laser-based therapies, plaque brachytherapy (radiation treatment), or in rare cases enucleation (removal of the eye).

Occular Tumors
TYPES OF TUMORS
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In general, tumors are either benign (not cancerous) or malignant (cancerous). They may occur in different parts of the eye and cause varying visual effects depending on the specific location involved. Intraocular tumors may be found on a routine eye exam, or may cause vision problems prompting those affected to seek care. Frequently patients are referred to a Colorado Retina specialist to determine if suspicious ocular lesions should be observed or treated. Once a patient is referred over, our tumor service is designed to help patients schedule appropriately for prompt evaluations.  The initial clinic visits usually require several hours as baseline imaging studies are needed and a clinical exam with a physician is performed.  For most, a specific diagnosis is quickly established, and a plan for follow-up is developed.

A newly identified intraocular tumor(or lesion) causes anxiety for many patients.  By definition intraocular lesions are found inside the eye, and usually are not visible without special eye exams.  Some intraocular lesions may be found on a routine eye exam even in patients without vision symptoms. Some intraocular lesions may cause visual disturbances depending on the specific location within the eye and relation to intraocular structures.

Malignant tumors are associated with cancer and can affect the whole body. For some, the malignant tumor of the eye may be the first sign of systemic cancer. Malignant tumors may start in the eye, such as ocular melanoma. Other malignant tumors are metastases, which have spread to the eye from cancer in another part of the body, such as breast, lung, GI tract, prostate or other sources. Lymphoma may also evolve within the eye.

Benign tumors are not harmful to one's health, and may be observed when not affecting vision. Regular observation is recommended as some benign conditions may undergo “neoplastic transformation” to become malignant. Some types of benign tumors may be associated with systemic conditions and further testing may be needed. Examples of benign tumors include nevus, osteoma, hemangioma, congenital hypertrophy of the retinal pigment epithelium (CHRPE), and others. Benign tumors of the eye are important to accurately diagnose and measure as they are sometimes associated with other types of medical conditions.  It is rare for these lesions to have the potential to turn into cancer.



TYPES OF BENIGN TUMORS / LESIONS

   1.    Choroidal nevus - is a focal growth of melanocytes in the choroid.  A wide variety of nevi exist, with differences in size, shape, and color. Baseline examinations are used as references for future exams.  A choroidal nevus is usually stable on serial exams; any changes in size or appearance may signal“neoplastic transformation” into a melanoma.

   2.    Congenital Hypertrophy of the Retinal Pigment Epithelium (CHRPE) is a benign condition thought to be present at birth, although usually detected in childhood or young adulthood.  These have a characteristic appearance and are usually located non-centrally with little if any effect on vision.  Extremely rare conversion to RPE adenocarcinoma has been described.

   3.    Choroidal hemangioma is a vascular lesion of abnormal tissue in the choroid. Depending on the location and propensity to accumulate sub-retinal fluid, choroidal hemangiomas may cause vision disturbances. A specialized form of laser-activated dye treatment known as Photodynamic therapy (PDT) may be effective in improving vision.  Choroidal hemangiomas which are growing may respond to radiation treatment (either external radiation or plaque brachytherapy). Choroidal hemangiomas can be associated with Sturge-Weber Syndrome.

   4.    Choroidal osteoma is an abnormality of the choroid in which localized calcium deposition disrupts the retinal function.  Sub-retinal neovascularization may occur and treatment can be useful in preserving vision.

   5.    Melanocytoma is a pigmented lesion usually found on the optic nerve head.  It does not grow, although the appearance may change during a normal posterior vitreous detachment (PVD), and should be monitored during this occurrence.  Neoplastic transformation into melanoma is rare.

   6.    Astrocytic Hamartoma is usually found in children and has a characteristic appearance, which distinguishes it from malignancies.

   7.    Vasoproliferative tumors, Coats disease, and eccentric disciform (PEHCR) are vascular lesions that can appear as growths in the retina or choroid.  Exams should be performed to exclude the diagnosis of Von-Hippel Lindau syndrome which is associated with systemic malignancies.  Proper diagnosis of these lesions allows for appropriate therapy when needed.

    

INTRAOCULAR TUMORS / OCULAR CANCERS
   1.    Choroidal melanoma is the most common primary cancer occurring in the adult eye.  Choroidal melanoma is diagnosed by the characteristic features observed with multimodal imaging and clinic exam.  Choroidal, ciliary body, and iris melanoma are the three forms of intraocular melanoma, and are known collectively as uveal melanoma.  The term “ocular melanoma” is the most broad as it includes uveal and conjunctival melanoma. All ocular melanomas may be able to spread to other parts of the body, a manifestation known as metastasis.  Uveal melanoma metastasis typically involves the liver.  Genetic mutations within the melanoma cells determine the ability to metastasize.  The genetic evaluation of melanomas by fine needle aspirate biopsy (FNAB) has become an international standard of care.

   2.    Choroidal metastasis
Cancers originating in other tissues of the body(e.g. breast, lung, GI tract, prostate or other sources) may metastasize to the eye.  Though many parts of the eye may be affected, usually the choroid is involved. The choroidal metastasis will commonly cause vision problems due to mass effects and fluid accumulations in the sub-retinal space.  Treatment is coordinated with oncologists and designed to eliminate the tumor in the eye while preserving or improving the vision.  In some cases a biopsy of the eye is important for diagnosis or treatment.

   3.    Lymphoma may also involve the eye structures and frequently will need specialized biopsies for diagnosis. Primary intraocular lymphoma (PIOL) commonly found in the vitreous is associated with central nervous system (CNS) lymphoma.  Choroidal lymphoma may have amore benign course.  

DIAGNOSTIC TESTING & IMAGING
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Most tumors will require periodic evaluations to monitor for change or evaluate response to treatment. The primary focus of the exam is to characterize the tumors in terms of size and shape and other qualities. Multiple imaging technologies are usually needed to create an accurate description of the tumor. This multimodal imaging can include specialized cameras, blood flow analysis, and ultrasound measurements. Due to the specialized testing needed exams may require two or three hours. Cancer patients will also require routine body imaging studies.

MULTIMODAL IMAGING of the eye includes:
   1.    Digital wide-field imaging -
a specialized camera system, scanning laser ophthalmoscopy (SLO), is used to create high-resolution images of the layers of inside wall the eye. This allows high contrast reproducible imaging of tumors, with precise descriptions of size and relationship to intraocular structures.  Some tumor features are best visualized in non-visible wavelengths.

   2.    Enhanced depth imaging (EDI) is a form ofOcular Coherence Tomography (OCT), is used to create precise 3-dimensional mapping of smaller tumors with resolution measured in microns (less than a ten-thousandth of an inch).  Automatic computerized comparisons are made with images taken on separate visits, and are able to detect minute changes in structures. This sensitivity is critical to determine if a tumor is growing, shrinking, or inert.

   3.    Wide-field angiography - The inside of the eye has two blood supplies, which can be interrogated separately using safe tracerdyes and specialized imaging cameras.  Fluorescein angiography allows evaluation of the retinal circulation, while ICG angiographyis used to visualize the choroidal circulation.   Commonly both will be used simultaneously to evaluate tumors.

   4.    Ultrasonography - Anterior and posterior ultrasound instruments are used to evaluate size and location of the tumors and describe their relation to other eye structures. Some tumors are “hidden” behind the iris and can only be accurately visualized and measured with anterior ultrasound.  Both forms of ultrasound can be easily performed in the clinic.

TREATMENT
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Treatment is designed to eradicate the tumor in the eye while preserving vision if possible. Fortunately, many treatment options are available including medications, injections, laser procedures, or even surgery. Benign tumors may be treated to improve vision. Malignant tumors are treated in coordination with other oncologists. Sometimes a biopsy is needed, requiring a trip to the operating room to obtain a tissue sample.

Several types of lasers can be performed in the clinic, including, argon, photodynamic therapy (PDT), and diode transpupillary thermotherapy (TTT) to destroy tumor tissue. Radiation treatment (e.g. plaque brachytherapy) is also routinely performed by our team of ocular specialists at Colorado Retina. When it is not possible to save the eye, removal of the eye (enucleation) is offered. We often coordinate care with other physicians including eye doctors, oncologists, radiologists, and proton centers.

Side effects of radiation treatment applied to the eye may take years to develop and include cataracts, glaucoma, and radiation retinopathy, depending on the location and size of the tumor and the radiation dose used. Treatment of the side effects of radiation is possible, and for many it prolongs useful vision.

If the intraocular tumor is determined to be cancerous, the next step of the work up is “staging” to determine if other parts of the body are affected. Systemic imaging ordered in accordance with current National Comprehensive CancerNetwork recommendation guidelines.  Usually this involves imaging studies directed at determining if metastasis is present.  These studies may include PET-CT, MRI, CT, or ultrasound, and are usually performed shortly after the first office visit, often at a facility near the patient’s home.  Patients are referred to an oncologist forco-management.

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Meet Your Specialists

Peter G. Hovland Headshot

Peter G. Hovland  |  MD, PHD

Leading our ocular oncology department is Dr. Peter Hovland, a renowned ophthalmologist, surgeon and founding partner at Colorado Retina Associates. Dr. Hovland and his team of trained specialists focus on the surgical and medical treatment of all types of intraocular tumors, from the most common to complex. Dr. Hovland has cared for thousands of patients from around the US with benign and malignant eye tumors, including ocular melanoma, choroidal or iris nevus, choroidal metastases and other vision threatening retinal conditions. Dr. Hovland is also actively involved in supporting the fight to find a cure for cure ocular melanoma, and currently acts as a grant reviewer for the Melanoma Research Foundation and the Ocular Melanoma Foundation.

Amanada Sisco Headshot

Amanda (Mason) Sisco  |  OSC/MA/CME

Amanda Sisco works in collaboration with Dr. Peter Hovland as Colorado Retina’s Supervisor of Oncology Services. Amanda has been with Colorado Retina for the past five years and works closely with our oncology patients to ensure they are well informed and cared for through the phases of testing, diagnosis, treatment and follow-up. Amanda was awarded the 2019 CURE OM Vision of Hope Award at the Melanoma Research Foundation’s 8th Annual Denver Gala for her advocacy work surrounding ocular melanoma patient support, treatment and care. Amanda is also co-chair of the Melanoma Research Foundation’s Treatment Center Collaborative Coalition.

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