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This section outlines treatments that are the standard of care (the best proven treatments available) for this specific type of tumor. When making treatment plan decisions, patients are also encouraged to consider clinical trials as an option. A clinical trial is a research study to test a new treatment to evaluate whether it is safe, effective, and possibly better than standard treatment. Your doctor can help you review all treatment options.
Many people with lacrimal gland tumor are treated by more than one specialist with more than one type of treatment. This is called a multidisciplinary team approach. For example, patients who receive radiation therapy to the eye may need to also talk with an ophthalmologist or plastic surgeon to make sure the eye can still function after treatment.
Patients should have a sense that their doctors have a coordinated plan of care and are communicating effectively with one another. If patients do not feel that the team is communicating effectively with them or each other about the goals of treatment and the plan of care, patients should discuss this with their doctors or seek additional opinions before treatment.
Descriptions of the most common treatment options for lacrimal gland tumor are listed below. Goals of treatment include complete removal of the tumor, as well as maintaining the health and vision of the patient's eye. Treatment options and recommendations depend on several factors, including the type and stage of cancer, possible side effects, whether one or both eyes are involved, whether the tumor is cancerous, and the patient's preferences and overall health.
Surgery is the removal of the tumor and surrounding tissue during an operation.
Surgery to the eye is quite common in the treatment of a lacrimal gland tumor. During surgery, the ophthalmologist will remove parts of the affected eye or, if necessary, the entire eye (called enucleation), depending on the size and spread of the tumor.
Possible side effects of eye surgery are similar to that of any surgery, including a risk of infection, problems with anesthesia (medication used during surgery to block awareness of pain), and pain.
Having an eye removed
Sometimes the only choice a doctor has in treating a lacrimal gland tumor is to remove the eye. Because of this visual loss, a person with one eye may have trouble with depth perception. Most people adjust to these differences.
Many people worry about what they will look like when they have an eye removed. The cosmetic surgery available today usually yields good cosmetic results. To fill the area left by the missing eye, the person is fitted for a prosthesis (artificial eye). The prosthesis will look and behave almost the same as a natural eye. For example, the artificial eye will move along with the person's remaining eye, just not as much as a natural eye moves. Family members may be able to tell that the eye is not real, but it is unlikely that strangers will know. If enucleation is required, talk with your doctor about a prosthesis; it may take many weeks for patients to receive the prosthesis. Also, ask about support services that may be available to you to help adjust to the loss of an eye.
Radiation therapy is the use of high-energy x-rays or other particles to kill cancer cells. It is most often used for lacrimal gland lymphoma. A doctor who specializes in giving radiation therapy to treat cancer is called a radiation oncologist. A radiation therapy regimen (schedule) usually consists of a specific number of treatments given over a specific time.
There are different types of radiation therapy. The most common type of radiation treatment is called external-beam radiation therapy, which is radiation given from a machine outside the body. Proton therapy (also called proton beam therapy) is a type of external-beam radiation therapy that uses protons rather than x-rays. At high energy, protons can destroy cancer cells.
Intensity modulated radiation therapy (IMRT) is another way to deliver external-beam radiation therapy. The intensity is varied to more precisely target the tumor, and therefore damages less surrounding healthy tissue than is possible with traditional radiation treatment. IMRT may also reduce the damage to nearby important organs.
The dosage of radiation used and the site and type of the tumor significantly affect the risks of side effects. Cataracts are a very common side effect of radiation therapy to the eye area. A cataract is opacity (a lack of transparency) of the lens or capsule of the eye. People with cataracts may have cloudy or foggy vision, have trouble seeing at night, and/or have problems with glare from the sun or bright lights. If the cataract is causing major problems with a person's eyesight, the cataract can be surgically removed.
Also, loss of eyelashes and/or a dry eye can occur with external-beam radiation therapy. Other side effects from radiation therapy may include fatigue, mild skin reactions, upset stomach, and loose bowel movements. Most side effects go away soon after treatment is finished.
Other side effects are possible but more uncommon. Radiation retinopathy is the development of abnormal blood vessels in the retina. Radiation optic neuropathy involves nerve damage in the eye. Neovascular glaucoma is a painful condition in which new blood vessels develop and block the regular release of fluid from the eye. If there is significant damage to the eye from radiation therapy, the eye may need to be removed.
Talk with your doctor about the risks and benefits of the different types of radiation therapy.
Chemotherapy is the use of drugs to kill tumor cells, usually by stopping the cancer cells' ability to grow and divide. Systemic chemotherapy is delivered through the bloodstream to reach tumor cells throughout the body. Chemotherapy is given by a medical oncologist, a doctor who specializes in treating a tumor with medication. A chemotherapy regimen (schedule) usually consists of a specific number of cycles given over a set period of time. A patient may receive one drug at a time or combinations of different drugs at the same time.
The side effects of chemotherapy depend on the individual and the dose used, but they can include fatigue, risk of infection, nausea and vomiting, loss of appetite, and diarrhea. These side effects usually go away once treatment is finished.
The medications used to treat cancer are continually being evaluated. Talking with your doctor is often the best way to learn about the medications prescribed for you, their purpose, and their potential side effects or interactions with other medications.
Immunotherapy (also called biologic therapy) is designed to boost the body's natural defenses to fight the tumor. It uses materials made either by the body or in a laboratory to bolster, target, or restore immune system function.
Rituximab (Rituxan) is the most common immunotherapy used in the treatment of a lacrimal gland tumor; it is also used for the treatment of non-Hodgkin lymphoma.
Treatment by type of lacrimal gland tumor
Below is an outline of the common treatments used depending on the type and stage of the lacrimal gland tumor. In addition to standard treatments, patients are encouraged to talk with their doctors about clinical trials that are open to them, no matter the type or stage of the tumor.
Lymphoma may be treated with external-beam radiation therapy, chemotherapy, immunotherapy, or a combination of these treatments. The chance of recovering from a lymphoma of the lacrimal gland is higher if only one eye is affected. The specific treatment for ocular lymphoma depends on whether other parts of the body are affected, so it is important to know the stage of the tumor.
Benign mixed epithelial tumor
The most common type of treatment for a benign mixed epithelial tumor is an excisional biopsy, where the tumor is removed surgically . The prognosis is more favorable if the tumor is completely removed.
Malignant mixed epithelial tumor
The most common treatment for a malignant mixed epithelial lacrimal gland tumor is the complete surgical removal of the tumor.
AdCC of the lacrimal gland
AdCC is an aggressive form of cancer, and the most common type of treatment for AdCC is a procedure called exenteration. In this procedure, the surgeon removes the lacrimal gland, eyeball, muscles, and all orbital contents and adjacent bone. A combination of chemotherapy and radiation therapy may also be used as part of the treatment plan. Treatment is most successful when the cancer has not spread.
A tumor and its treatment often cause side effects. In addition to treatment to slow, stop, or eliminate the tumor, an important part of care is relieving a person's symptoms and side effects. This approach is called palliative or supportive care, and it includes supporting the patient with his or her physical, emotional, and social needs.
Palliative care can help a person at any stage of illness. People often receive treatment for the tumor and treatment to ease side effects at the same time. In fact, patients who receive both often have less severe symptoms, better quality of life, and report they are more satisfied with treatment.
Before treatment begins, talk with your health care team about the possible side effects of your specific treatment plan and supportive care options. And during and after treatment, be sure to tell your doctor or another health care team member if you are experiencing a problem, so it is addressed as quickly as possible.
Recurrent lacrimal gland tumor
A remission is when a tumor cannot be detected in the body and there are no symptoms. This may also be called “no evidence of disease” or NED.
A remission can be temporary or permanent. This uncertainty leads to many survivors feeling worried or anxious that the tumor will come back. While many remissions are permanent, it's important to talk with your doctor about the possibility of the tumor returning. Understanding the risk of recurrence and the treatment options may help you feel more prepared if the tumor does return.
If the tumor does return after the original treatment, it is called a recurrent lacrimal gland tumor. It may come back in the same place (called a local recurrence), nearby (regional recurrence), or in another place (distant recurrence).
When this occurs, a cycle of testing will begin again to learn as much as possible about the recurrence. After testing is done, you and your doctor will talk about your treatment options. Often the treatment plan will include the therapies described above (such as surgery, chemotherapy, and radiation therapy) but may be used in a different combination or given at a different pace. Usually, surgery is the first option for treating a recurrence if it hasn't been done already. If there is no standard drug available, your doctor may suggest participation in a phase I clinical trial, where new drugs are tested to find out the safety and effective dose of the drug (although not necessarily whether the drug is effective for treating this type of tumor).
People with recurrent cancer often experience emotions such as disbelief or fear. Patients are encouraged to talk with their health care team about these feelings and ask about support services to help them cope.
Metastatic lacrimal gland tumor
If a tumor has spread to another location in the body, it is called metastatic lacrimal gland tumor. Patients with this diagnosis are encouraged to talk with doctors who are experienced in treating this stage of cancer, because there can be different opinions about the best treatment plan.
Your health care team may recommend a treatment plan that includes a combination of surgery, radiation therapy, immunotherapy, and chemotherapy. Supportive care will also be important to help relieve symptoms and side effects.
For many patients, a diagnosis of metastatic cancer can be very stressful and, at times, difficult to bear. Patients and their families are encouraged to talk about the way they are feeling with doctors, nurses, social workers, or other members of the health care team. It may also be helpful to talk with other patients, including through a support group.
If treatment fails
Recovery from a lacrimal gland tumor is not always possible. If treatment is not successful, the disease may be called advanced or terminal cancer.
This diagnosis is stressful, and this is difficult to discuss for many people. However, it is important to have open and honest conversations with your doctor and health care team to express your feelings, preferences, and concerns. The health care team is there to help, and many team members have special skills, experience, and knowledge to support patients and their families. Making sure a person is physically comfortable and free from pain is extremely important.
Palliative care given toward the end of a person's life is called hospice care. You and your family are encouraged to think about where you would be most comfortable: at home, in the hospital, or in a hospice environment. Nursing care and special equipment can make staying at home a workable alternative for many families.
Doctors and scientists are always looking for better ways to treat patients with a lacrimal gland tumor. To make scientific advances, doctors create research studies involving people, called clinical trials. Many clinical trials are focused on new treatments, evaluating whether a new treatment is safe, effective, and possibly better than the current (standard) treatment. These types of studies evaluate new drugs, different combinations of existing treatments, new approaches to radiation therapy or surgery, and new methods of treatment.
Patients who participate in clinical trials are often among the first to receive new treatments before they are widely available. However, there is no guarantee that the new treatment will be safe, effective, or better than a standard treatment.
There are also clinical trials that study new ways to ease symptoms and side effects during treatment and managing the late effects that may occur after treatment. Talk with your doctor about clinical trials regarding side effects. In addition, there are ongoing studies about ways to prevent the disease.
Patients decide to participate in clinical trials for many reasons. For some patients, a clinical trial is the best treatment option available. Because standard treatments are not perfect, patients are often willing to face the added uncertainty of a clinical trial in the hope of a better result. Other patients volunteer for clinical trials because they know that these studies are the only way to make progress in treating lacrimal gland tumors. Even if they do not benefit directly from the clinical trial, their participation may benefit future patients with lacrimal gland tumor.
Sometimes people have concerns that, by participating in a clinical trial, they may receive no treatment by being given a placebo or a “sugar pill.” The use of placebos in cancer clinical trials is rare. When a placebo is used in a study, it is done with the full knowledge of the participants.
To join a clinical trial, patients must participate in a process known as informed consent. During informed consent, the doctor should list all of the patient's options, so that the person understands how the new treatment differs from the standard treatment. The doctor must also list all of the risks of the new treatment, which may or may not be different from the risks of standard treatment. Finally, the doctor must explain what will be required of each patient in order to participate in the clinical trial, including the number of doctor visits, tests, and the schedule of treatment.
Patients who participate in a clinical trial may stop participating at any time for any personal or medical reason. This may include that the new treatment is not working or there are serious side effects. Clinical trials are also closely monitored by experts who watch for any problems with each study. It is important that patients participating in a clinical trial talk with their doctor and researchers about who will be providing their treatment and care during the clinical trial, after the clinical trials ends, and/or if the patient chooses to leave the clinical trial before it ends.
Coping with Side Effects
Fear of treatment side effects is common after a diagnosis of a lacrimal gland tumor, but it may help to know that preventing and controlling side effects is a major focus of your health care team. This is called palliative or supportive care, and it is an important part of the overall treatment plan, regardless of the stage of disease.
Common side effects from each treatment option for a lacrimal gland tumor are described in detail within the Treatment section. Side effects depend on a variety of factors, including the tumor's stage, the length and dosage of treatment(s), and your overall health.
Before treatment begins, talk with your doctor about possible side effects of each type of treatment you will be receiving. Ask which side effects are most likely to happen, when they are likely to occur, and what can be done to prevent or relieve them. And, ask about the level of caregiving you may need during treatment and recovery, as family members and friends often play an important role in the care of a person with a lacrimal gland tumor.
In addition to physical side effects, there may be psychosocial (emotional and social) effects as well. Patients and their families are encouraged to share their feelings with a member of their health care team who can help with coping strategies.
During and after treatment, be sure to tell the health care team about the side effects you experience, even if you feel they are not serious. Sometimes, side effects can last beyond the treatment period, called a long-term side effect. A side effect that occurs months or years after treatment is called a late effect. Treatment of both types of effects is an important part of survivorship care.
After treatment for a lacrimal gland tumor ends, talk with your doctor about developing a follow-up care plan. This plan may include regular physical examinations and/or medical tests to monitor your recovery for the coming months and years. ASCO offers treatment summary forms to help keep track of the treatment you received and develop a survivorship care plan once treatment is completed.
People recovering from a lacrimal gland tumor are encouraged to follow established guidelines for good health, such as maintaining a healthy weight, not smoking, eating a balanced diet, and having recommended cancer screening tests. Talk with your doctor to develop a plan that is best for your needs. Moderate physical activity can help rebuild your strength and energy level. Your doctor can help you create an appropriate exercise plan based upon your needs, physical abilities, and fitness level.
Doctors are working to learn more about lacrimal gland tumors, ways to prevent them, how to best treat them, and how to provide the best care to people diagnosed with this disease. The following areas of research may include new options for patients through clinical trials. Always talk with your doctor about the diagnostic and treatment options best for you
Radioimmunotherapy. Radioimmunotherapy is a treatment that combines the effectiveness of radiation therapy with immunotherapy. A radioactive material is attached to a protein that identifies the tumor cells as targets, and then a monoclonal antibody carries the radiation to the target cells. The radioactivity kills the cells with which it comes in contact. The most common form of radioimmunotherapy that is being researched is ibritumomab tiuxetan (Zevalin), which is used in the treatment of lymphoma.
Improved radiation therapy. Many hospitals and cancer centers offer methods that better focus radiation therapy to the tumor, helping reduce damage to the rest of the eye. One of these methods, called intensity-modulated proton therapy (IMPT), is being used for some types of eye cancer.
Chemotherapy. Researchers continue to explore new drugs and combinations of drugs that may benefit people with a lacrimal gland tumor.
Supportive care. Clinical trials are underway to find better ways of reducing symptoms and side effects of current lacrimal gland tumor treatments in order to improve patients' comfort and quality of life.