Ocular Oncology
Ocular oncology encompasses the management of all tumors involving the
eye, eyelid, orbit and lacrimal glands. Ocular tumors affect adults
and children,
and tumors may occur in one eye or may involve both eyes.
What are some common ocular cancers?
Some common ocular cancers include choroidal melanoma, choroidal hemangioma,
retinoblastoma, eyelid tumor, conjunctival tumor and lymphoma/leukemia.
Choroidal Melanoma
Choroidal melanoma is the most common primary intraocular (occurring
inside the eye) tumor in adults. It arises from the pigmented cells of the
choroid of the eye and is not a tumor that started somewhere else and
spread to the eye.
A choroidal melanoma is malignant, meaning that the cancer may
metastasize and eventually spread to other parts of the body. Because
choroidal melanoma is intraocular and not usually visible, patients with
this disease often do not recognize its presence until the tumor grows to
a size that impairs vision by obstruction, retinal detachment, hemorrhage,
or other complication. Pain is unusual, except with large tumors. Periodic
retinal examination through a dilated pupil is the best means of early
detection.
Cutting out the tumor and leaving the rest of the eye is not routinely
advised for this type of cancer. Opening the eye during surgery would
allow the tumor cells to float around into the spaces around the eye,
which could spread cancer cells to other parts of the eye. In addition,
some studies have shown that up to 50% of choroidal melanomas invade the
sclera. Therefore if the tumor is removed from the eye there is a high
possibility that cancer cells will remain within the sclera. Lastly, many
eyes do not tolerate this procedure and severe complications may occur
such as detachments of the retina, hemorrhages, and recurrence of the
tumor which may result in having to remove the eye anyway.
Treatment recommendations for choroidal melanoma usually are based on
the size of the tumor. Small suspicious melanomas usually are closely
watched for evidence of growth before treatment is recommended.
Medium-sized tumors may be treated with either radioactive plaque
therapy or enucleation (removal) of the eye. The
Collaborative
Ocular Melanoma Study (COMS), supported by the
National Eye Institute
of the National Institutes
of Health, has documented equal success rates for plaque
radiation therapy or enucleation for
preventing the spread of cancer. Large-size tumors usually are
best treated by enucleation. This is because the amount of radiation
required
to treat the tumor is too much for the eye to tolerate. The COMS
study found no benefit to large-size tumor patients having radiation
therapy
prior to enucleation.
What advantage does Bascom Palmer Eye Institute offer a patient with choroidal melanoma?
The Ocular Oncology Service at the Bascom Palmer Eye Institute is one of
the premier international sites for management of ocular malignancy. Bascom
Palmer Eye Institute and the University of Miami allow a focused
translational approach to patients with ocular oncologic process with a
particular focus on posterior uveal malignant melanoma (choroidal/ciliary
body melanoma), choroidal metastatic lesions, retinoblastoma, lymphoma,
leukemia, and vascular tumors. Patients with choroidal melanoma undergoing
evaluation and treatment at the Bascom Palmer Eye Institute have access to
the most advanced diagnostic imaging testing and treatment modalities
available currently in the United States. Accuracy of diagnosis at the
Bascom Palmer Eye Institute has exceeded 99% in evaluation of complex ocular
malignancies, utilizing this advanced technology. Bascom Palmer Eye Institute
has an ongoing focus on the eradication of intraocular tumor associated with
conservation of the globe. This treatment approach allows the patient to
maintain their eye with visual function in the setting of definitive
treatment for their melanoma. Advanced treatment options include focused
radiotherapy, laser hyperthermia, combined application treatments including
radiotherapy, hyperthermia, and surgical resection; all are options for
patients for treatment at the Bascom Palmer Eye Institute. Currently, local
tumor control rates for patients with choroidal melanoma are exceeding 98.5%
in long-term follow-up of all patients treated within the institute's Ocular
Oncology Service. A long-term commitment to aggressive patient follow-up is
also inherent within the Ocular Oncology Service. Patients are typically
followed at the Institute at a 4 to 6 month interval, including comprehensive
ocular imaging with both wide-field high-resolution photographs and advanced
high-resolution echography. The integration of aggressive advanced imaging
with the comprehensive ophthalmic evaluation allows for close serial follow-up
to document tumor control and absence of tumor related complications, insuring
the greatest likelihood for maintenance of vision and ocular function. The Bascom
Palmer Eye Institute integrates its patient evaluation with the Departments of
Radiation Oncology and Medical Oncology at the University of Miami Sylvester
Comprehensive Cancer Center, allowing for single comprehensive evaluation from
a focused ophthalmic perspective and a general systemic approach to the overall
management of the patient with primary choroidal melanoma.
What research is being conducted on choroidal melanoma in the United States?
Choroidal melanoma remains the most common ocular primary malignancy in the
United States. Intensive research efforts at the Bascom Palmer Eye Institute
and elsewhere continue to address several key questions in the diagnosis,
treatment, and long-term follow-up of patients with choroidal melanoma.
Diagnostic research continues to focus on enhanced imaging of primary ocular
tumors and the ability to differentiated primary malignant from a nonmalignant
tumor. Systemic staging research is focused on early detection of micrometastatic
disease (microscopic tumor spread outside of the eye).
Advanced treatment research has targeted improved function of the tumor containing eye utilizing adjunctive
treatments including radiotherapy. Experimental treatment protocols have focused
on patients developing metastatic disease (gross tumor spread beyond the eye)
and have included immunotherapy, direct resection, chemoembolization of isolated
disease, and targeted perfusion.
What research is being conducted on choroidal melanoma at Bascom Palmer Eye Institute?
The ocular oncology center of the Bascom Palmer Eye Institute, University of Miami Miller School of Medicine has focused on targeted tumor imaging with high resolution,
digital ultrasound, digital widefield photography and combined modality imaging to
assess unique tumor characteristics, document tumor status, and coordinate tumor
follow-up for stability of response and to detect tumor related complications.
Treatment related research has targeted combined and adjunctive therapy to achieve
total tumor control. Clinical research at BPEI has documented local tumor control
rates of greater than 98% for targeted radiotherapy. Intraoperative tumor localization
and assisted positioning for radiotherapy, particularly utilizing high resolution
echography, appear to be associated with significant improvement in local tumor
control. Clinical outcomes research has documented excellent survival outcomes for
patients undergoing advanced treatment and follow-up.
Basic and translational research studies at Bascom Palmer Eye Institute have
utilized advanced tumor culture techniques to assess predictors of survival and tumor
control in patients with aggressive, large choroidal melanoma and atypical clinical
presentations.
Choroidal Hemangioma
A hemangioma is a tumor comprised of blood vessels and can grow within
the choroid, the blood vessel layer beneath the retina. Choroidal
hemangiomas are not cancers and never metastasize. However, if the
hemangioma is located in the area of central vision of the eye it can leak
fluid that causes a retinal detachment and visual function may be
affected.
Many choroidal hemangiomas can be safely monitored by your eye doctor
without the need of further treatment. Photographs can be used to document
evidence of growth or leakage and the need for treatment. Treatment
options may include laser photocoagulation to
decrease the amount of fluid leakage or low doses of external
beam radiation therapy.
Choroidal Metastasis
Malignant tumors from other parts of the body can spread in and around
the eye. Metastatic cancers that appear in the eye usually come from a
primary cancer of the breast in women and the lungs in men. Other, less
common, sites of origin include the prostate, kidneys, thyroid, and the
gastrointestinal tract. Blood cell tumors (lymphomas and leukemia) also
can spread to the eye. The care of patients with metastasis to the eye
should be coordinated between the eye cancer specialist, medical
oncologist, and radiation oncologist. Treatment options may include
chemotherapy, external beam
radiation therapy, or, more rarely, enucleation.
Choroidal Nevus
Like a raised freckle on the skin, a nevus can occur inside the eye.
And, like a skin nevus, a choroidal freckle can become malignant, so
should be closely monitored. A choroidal nevus should be examined by an
ophthalmologist every four to six months to check if the pigmentation or
size of the nevus has changed. In most cases, the only treatment
recommended is close observation and monitoring by an ocular oncologist.
Conjunctival Tumors
Conjunctival tumors are malignant cancers that grow on the outer surface
of the eye. The most common types of conjunctival tumors are squamous cell
carcinoma, malignant melanoma, and lymphoma. Squamous cell carcinomas
rarely metastasize, but can invade the area around the eye into the orbit
and sinuses. Malignant melanomas can start as a nevus (freckle) or can
arise as newly formed pigmentation. Lymphoma of the eye can be a sign of
systemic lymphoma or be confined to the conjunctiva.
Both squamous cell carcinomas and malignant conjunctival melanomas
should be removed. Most small conjunctival tumors can be photographed and
followed for evidence of growth prior to treatment. Small tumors can be
completely removed surgically. In other instances, cryotherapy (freezing
therapy) may be necessary or chemotherapy eye drops may be used to treat
the entire surface of the eye.
Eyelid Tumors
Tumors of the eyelid may be benign cysts, inflammation, or malignant
skin cancers. The most common type of eyelid cancer is basal cell
carcinoma. Other common eyelid cancers include squamous cell carcinoma
and sebaceous gland carcinoma. Most basal cell carcinomas can be removed with surgery. If left
untreated, these tumors can grow around the eye and into the orbit,
sinuses and brain. A simple biopsy can determine if an eyelid tumor is
malignant. Malignant tumors are completely removed and the eyelid is
repaired using plastic surgery techniques. This usually results in a complete
cure of the eyelid cancer. Additional cryotherapy
(freezing-therapy) and radiation therapy sometimes are
required after surgery.
Iris Tumors
Tumors can grow within and behind the iris. Though many iris tumors are
cysts or a nevus, malignant melanomas can occur in this area. Most
pigmented iris tumors do not grow. They are photographed and monitored
with a special slit lamp and high frequency ultrasound to establish a
baseline for future comparisons. When an iris tumor is documented to grow,
treatment is recommended. Most small iris melanomas can be surgically
removed. Radiation plaque therapy or
enucleation may be considered for larger iris
tumors.
Lymphoma/Leukemia
Lymphoma tumors can appear in the eyelid tissue, tear ducts and the eye
itself. In most patients with large cell non-Hodgkin's lymphoma, the
disease is confined to the eye and central nervous system. In these
patients, symptoms appear in the eye an average of two years before they
are seen elsewhere. The disease itself as well as treatment, which may
include external beam radiation, chemotherapy,
or both (chemoradiation) to the central nervous system, can affect visual
functioning.
Orbital Tumors
Tumors and inflammations can occur behind the eye. These tumors often
push the eye forward causing a bulging of the eye called proptosis. The
most common causes of proptosis are thyroid eye disease and lymphoid
tumors. Other tumors include hemangiomas (blood vessel tumors), lacrimal
(tear) gland tumors, and growths that extend from the sinuses into the
orbit. Though CT scans, MRI's and
ultrasounds help in determining the probable
diagnosis, most orbital tumors are diagnosed by a biopsy.
When possible, orbital tumors are totally removed. If they cannot be
removed or if removal will cause too much damage to other important
structures around the eye, a piece of tumor may be removed and sent for
evaluation. If a tumor cannot be removed during surgery, most orbital
tumors can be treated with external beam radiation
therapy. Orbital lymphomas are usually biopsied. After a complete work-up
by an oncologist, treatment with radiation therapy is usually indicated.
Chemotherapy may be needed if the lymphoma is found to involve other areas of the
body. This is performed in conjunction with a hematologist/oncologist.
Certain rare orbital tumors may require removal of the eye
and orbital contents. In certain cases, orbital radiotherapy may be used
to treat any residual tumor.
How is eye cancer diagnosed?
A retinal oncologist (an eye cancer specialist) can determine if you
have an eye cancer by performing a complete clinical examination. The
examination may include asking questions about your medical history,
examining both eyes, looking into the eye at the tumor, doing an
ultrasound examination, and obtaining specialized photographs. Biopsy,
which is often indicated to diagnose tumors in other parts of the body, is
rarely needed with eye cancer. Though occasionally necessary, biopsies are
usually avoided because they require opening the eye which risks spreading
of the tumor cells.
Eye Examination
Your ophthalmologist may be able to recognize an eye cancer by its
appearance, including the degree of pigmentation of the tumor, its shape
and location, and by other features. Unlike tumors in other parts of the
body, many eye cancers, including choroidal melanoma, may be directly
visible through the "window" provided by the pupil.
Ultrasound (Echography)
| During an ultrasound examination, sound waves are
directed toward the tumor by a small probe placed on the eye.
The patterns made by
reflection of the sound waves help to confirm that tumors are
present. Ultrasound can determine if there is extraocular involvement
(if the
tumor has spread outside the eye) and helps to determine the
thickness or height of the tumor. Black and white pictures
of the ultrasound
images may be taken for your physician to review. |
|
Photography
There are two types of special photographs ophthalmologists use to
assist in diagnosis: fluorescein angiography and fundus photographs.
- In fluorescein angiography, a special dye is injected into a
vein in the arm. As the dye passes through the blood vessels in the back
of the eye, this allows for a view of the circulation of the retina and
the layers beneath the retina, highlighting any abnormalities. Although
fluorescein angiography is not diagnostic, it is useful to exclude other
possible disorders.
- The fundus of the eye includes the retina, macula, fovea, optic disc
and retinal vessels. In fundus photography, the inner lining of
the eye is photographed with specially designed cameras through the
dilated pupil. This is a non-invasive and painless procedure that
produces a sharp view of the retina, the optic nerve and the retinal
vessels.
Additional Evaluations
Your doctor may request that you have a complete physical examination
and specific tests depending upon what he sees inside your eye. Tests may
include magnetic resonance imaging (MRI), a computerized tomography (CT)
scan, chest x-ray, and complete blood count.
- A Computerized Tomography (CT) scan involves a series of
X-ray images that provide a very clear picture of the eyes, the
surrounding tissue and the brain. Unlike an ordinary X-ray machine,
which takes one picture at a time, the scanner takes a number of small
pictures as it rotates around the patient.
- Magnetic Resonance Imaging (MRI) uses magnetic fields and
radio waves linked to a computer to create pictures of areas
inside the body. Because MRI can "see" through bone, it can
provide clearer pictures of a tumor located near bone and in
the orbit.
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What are the current treatment options?
Your doctor will recommend treatment based on your medical history and
the findings from the eye examination. It is not always necessary to treat
all eye cancers immediately. If a tumor is very small or very slow
growing, sometimes the doctor will closely monitor the tumor. If there are
any concerns, then treatment can be started. Treatment usually is
recommended when your physician determines that the tumor shows evidence
of growth or if there is the possibility of spreading to other parts of
the body if left untreated.
Chemotherapy
Although it is rarely used for eye cancer, chemotherapy is the most
common type of treatment for many other types of cancer. Chemotherapy is
the treatment of disease by means of drugs that have a specific toxic
effect upon the cancer cells. Chemotherapy selectively destroys cancerous
tissue.
There are many chemotherapeutic drugs available. Each type of drug has
potential side effects such as skin problems, nausea, vomiting, and
infections. Chemotherapy sometimes is recommended for choroidal
metastasis, conjunctival tumors and lymphoma.
Cryotherapy
Cryotherapy is the use of low temperatures to treat disease. Cryotherapy
is applied under local anesthesia. The goal of cryotherapy is to freeze
the malignant tissues in order to stimulate inflammation and scarring of
this tissue. Cryotherapy may be recommended for conjunctival or eyelid
tumors.
External Beam Radiation Therapy
Radiation therapy uses high-energy radiation from x-rays and other
sources to kill cancer cells and shrink tumors. Radiation that comes from
a machine outside the body is called external-beam radiation therapy as
opposed to radiation that is administered by placing a radiation plaque
over or very near the tumor (internal radiation therapy or brachytherapy).
External beam radiation therapy may be recommended for some choroidal
metastasis, eyelid tumors, choroidal hemangiomas, lymphomas and orbital
tumors.
Radiation Plaque Therapy (Brachytherapy)
Radiation plaque therapy is the most commonly used "eye-sparing"
treatment for choroidal melanoma. A radioactive plaque is a small, gold
covered, dish-shaped device that contains a radioactive source. Standard
low-energy radioactive eye-plaques contain rice-sized radiation seeds that
emit low energy photons. The gold coat of the plaque helps to aim the
radiation photons directly at the tumor and decrease radiation damage to
surrounding tissues. As the cells die, the tumor shrinks, although it
usually does not disappear entirely. Radiation plaque therapy may be
recommended for choroidal melanomas or iris melanomas.
Eye plaques are custom made to the dimensions of the tumor, usually
ranging in size from about 12 to 22 mm. in diameter (about the size of a
quarter). Careful calculations determine how long the plaque must remain
in place to give the tumor the proper amount of radiation. |
|
Surgical placement of the plaque lasts about an hour and usually is
performed under local anesthesia. During surgery, an incision is made in
the conjunctiva and the radioactive plaque is sutured to the sclera,
outside of the eye, over the tumor. The conjunctiva is then sewn back over
the plaque. Patients remain in the hospital for about three to five days
at which time the plaque is surgically removed.
Most patients have no problems associated with plaque surgery. As with
any ocular surgery, there can be secondary complications such as retinal
detachments, hemorrhages, or infections. There are also risks associated
with anesthesia.
The effects of radiation on the tumor typically are first evident three
months after treatment. Eventually, eye melanomas shrink to about 40% of
their pretreatment size. After successful treatment, although the tumors
rarely completely disappear, they are considered to be inactive.
After radioactive plaque treatment, many patients note some dryness and
irritation of the eye. In some instances, eyelashes may be permanently
lost. In rare instances, the outside layer of the eye (sclera) may become
very thin. Occasionally, prolonged redness, irritation, or infection may
occur. Some patients may experience double vision, which can last a few
days or several months. Radiation plaque therapy may cause eventual
blurring, dimming, or rarely a total loss of vision in the treated eye.
Plaque radiation does not affect the vision in the other eye. The amount
of vision loss depends on what your vision was before treatment, how close
the tumor is to the area of central vision of the eye, and how sensitive
your tissues are to radiation. Most people maintain some central vision,
and almost all retain peripheral vision.
Enucleation
Enucleation is the surgical removal of the eye, leaving eye muscles
and the contents of the eye socket intact. The eyelids, lashes,
brow and surrounding skin all remain.
This procedure is done when there is no other way to remove the cancer
completely from the eye. Unfortunately, loss of vision for the
eye removed is permanent because an eye cannot be transplanted. The eye
is removed,
and a spherical implant made of coral or hydroxyapatite is placed
into the orbit. This allows the blood vessels to grow into the porous
coral
material. Also, porous polyethylene implants are used. The muscles
that help give movement to the eye are then sutured to the implant, which
will allow for some movement of the prosthesis. |
The eye is surrounded by bones; therefore, it is much easier to tolerate
removal of an eye as compared to the loss of other organs. After a healing
period of approximately five weeks, a temporary ocular prosthesis (plastic-eye) is inserted. The
prosthesis is a plastic shell painted to match the other eye. It is
inserted under the eyelid, much like a big contact lens. After a final
prosthetic fitting most patients are happy with the way they look, and say
others can't even tell they have vision in only one eye.
After enucleation, there is reduced visual field on the side of the body
when looking straight ahead, and there is a loss of depth perception. Many
of the skills of depth perception can be relearned and with time, almost
all patients are able to do all the things they used to do before losing
their eye. A few people who did very well with only one eye include:
President Theodore Roosevelt, Israeli military leader Moshe Dayan,
Congressman Morris Udall, entertainers Sammy Davis Jr. and Sandy Duncan,
actor Peter Falk,
painter Edgar Degas, aviator Wiley Post, inventor Guglielmo Marconi and
British naval hero Horatio Nelson.
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Who are the Ocular Oncology
Specialists at Bascom Palmer Eye Institute?
Thomas E. Johnson, M.D.
Wendy W. Lee, M.D., M.S.
Timothy G. Murray, M.D., M.B.A., F.A.C.S.
David T. Tse, M.D.
Other Ocular Oncology Resources
American Academy of Ophthalmology
American Cancer Society
Collaborative Ocular Melanoma Study
Eye Cancer Network
Eye
Resources on the Internet
National Cancer Institute
National Eye
Institute
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