The lacrimal glands are the glands that secrete tears and are located above and to the side of the eye. When lacrimal gland cells become abnormal and start to multiply, they form a growth of tissue, called a tumor. A lacrimal gland tumor can be benign (noncancerous) or malignant (cancerous). There are four major types:
Benign mixed epithelial tumor. A benign mixed epithelial tumor is a noncancerous tumor that does not spread to other parts of the body, but will continue to grow if left untreated. This type of tumor begins in the cells that line the lacrimal gland.
Malignant mixed epithelial tumor. A malignant mixed epithelial tumor begins in the cells that line the lacrimal gland. If left untreated, it will spread to other parts of the body.
Lymphoma. Lymphoma can involve various structures of the eye; however, the conjunctiva (the mucous membrane lining the inner surfaces of the eyelids and the outer surface of the white of the eye) and lacrimal gland are the most common. Most ocular (eye-related) lymphoma is non-Hodgkin lymphoma, and may be associated with systemic or central nervous system lymphoma. Read the Cancer.Net Guide to Non-Hodgkin Lymphoma for more information.
Adenoid cystic carcinoma (AdCC) of the lacrimal gland. AdCC is a rare form of adenocarcinoma, which is a broad term covering any cancer arising from glandular tissues. An AdCC tumor is characterized by a distinctive pattern, in which bundles of epithelial cells surround and/or infiltrate ducts or glandular structures within the organ. When an AdCC tumor of the lacrimal gland grows, it commonly pushes the eye forward and causes it to bulge, a condition called proptosis. Another characteristic is pain, due to local nerves being invaded by the tumor.
Statistics
A lacrimal gland tumor is rare. Malignant epithelial tumors of the lacrimal gland account for 2% of all orbital (eye socket) tumors. AdCC is the most frequent epithelial orbital cancer, accounting for approximately 50% of malignant lacrimal gland tumors and 25% of all lacrimal gland tumors.
The five-year survival rate (the percentage of people who survive at least five years after the cancer is detected, excluding those who die from other disease) of AdCC is estimated to be 50%.
Cancer survival statistics should be interpreted with caution. It is not possible to tell a person how long he or she will live with a lacrimal gland tumor. Because the survival statistics are measured in five-year (or sometimes one-year) intervals, they may not represent advances made in the treatment or diagnosis of this cancer.
A risk factor is anything that increases a person's chance of developing cancer. Some risk factors can be controlled, such as smoking, and some cannot be controlled, such as age and family history. Although risk factors can influence the development of cancer, most do not directly cause cancer. Some people with several risk factors never develop the disease, while others with no known risk factors do. However, knowing your risk factors and communicating with your doctor can help guide you in making wise lifestyle and health-care choices.
Age. A lacrimal gland tumor occurs more frequently in people in their 30s.
Prior history of lymphoma. People who have a history of lymphoma are at higher risk for developing a periocular (around the eye) lymphoma.
Incomplete removal of prior benign tumor. If a noncancerous lacrimal gland tumor was not completely removed, there is a higher risk of a malignant lacrimal gland tumor occurring. Therefore, a careful, post-surgical assessment is recommended for people having this type of surgery.
People with a lacrimal gland tumor may experience the symptoms described below. Sometimes people with a lacrimal gland tumor do not show any of these symptoms. Or, these symptoms may be similar to symptoms of other medical conditions. If you are concerned about a symptom on this list, please talk with your ophthalmologist (a medical doctor who specializes in eye care).
Vision problems, such as blurry vision
Pain in or around the eye
A fullness of the eyelid, or a mass that can be felt on the eyelid
Doctors use many tests to diagnose cancer and determine if it has metastasized (spread). Some tests may also determine which treatments may be the most effective. For most types of cancer, a biopsy is the only way to make a definitive diagnosis of cancer. If a biopsy is not possible, the doctor may suggest other tests that will help make a diagnosis. Imaging tests may be used to find out whether the cancer has metastasized. Your doctor may consider these factors when choosing a diagnostic test:
Age and medical condition
The type of cancer
Severity of symptoms
Previous test results
The following tests may be used to diagnose a lacrimal gland tumor:
Biopsy. A biopsy removes a small amount of tissue for examination. The sample is analyzed by a pathologist (a doctor who specializes in interpreting laboratory tests and evaluating cells, tissues, and organs to diagnose disease). Other tests can suggest that cancer is present, but only a biopsy can make a definite diagnosis. The type of biopsy performed will depend on the location of the cancer. In an incisional biopsy, the surgeon cuts into the tumor and removes a sample of tissue. In an excisional biopsy, used more commonly for benign mixed epithelial tumors, the surgeon removes the entire tumor. A fine needle biopsy removes a small amount of tissue for examination under a microscope by inserting a needle directly into the tumor to extract cells. The use of fine needle biopsy for a lacrimal gland tumor remains controversial; talk with your doctor for more information.
Computed tomography (CT or CAT) scan. A CT scan creates a three-dimensional picture of the inside of the body with an x-ray machine. A computer then combines these images into a detailed, cross-sectional view that shows any abnormalities or tumors.
Magnetic resonance imaging (MRI). An MRI uses magnetic fields, not x-rays, to produce detailed images of the body.
Positron emission tomography (PET) scan. In a PET scan, radioactive sugar molecules are injected into the body. Cancer cells absorb sugar more quickly than normal cells, so they light up on the PET scan. PET scans are often used to complement information gathered from CT scan, MRI, and physical examination.
Bone scan. A bone scan uses a radioactive tracer to look at the inside of the bones. The tracer is injected into a patient's vein. It collects in areas of the bone and is detected by a special camera. Healthy bone appears gray to the camera, and areas of injury, such as those caused by cancer, appear dark.
Staging is a way of describing a tumor, such as where it is located, if or where it has spread, and if it is affecting the functions of other organs in the body. Doctors use diagnostic tests to determine the cancer's stage, so staging may not be complete until all the tests are finished. Knowing the stage helps the doctor to decide what kind of treatment is best and can help predict a patient's prognosis (chance of recovery). There are different stage descriptions for different types of cancers.
One tool that doctors use to describe the stage of a lacrimal gland tumor is the TNM system. This system uses three criteria to judge the stage of the cancer: the tumor itself, the lymph nodes around the tumor, and if the tumor has spread to the rest of the body. The results are combined to determine the stage of cancer for each person. There are five stages: stage 0 (zero) and stages I through IV (one through four). The stage provides a common way of describing the cancer so doctors can work together to plan the best treatments.
TNM is an abbreviation for tumor (T), node (N), and metastasis (M). Doctors look at these three factors to determine the stage of cancer:
How large is the primary tumor and where is it located? (Tumor, T)
Has the tumor spread to the lymph nodes? (Node, N)
Has the cancer metastasized to other parts of the body? (Metastasis, M)
Tumor. Using the TNM system, the "T" plus a letter and/or number (0 to 4) is used to describe the size and location of the tumor. Some stages are also divided into smaller groups that help describe a patient's condition in more detail. Specific tumor stage information is listed below:
TX: The primary tumor cannot be evaluated.
T0: There is no tumor.
T1: The tumor is 2.5 centimeters (cm) or smaller and is limited to the lacrimal gland.
T2: The tumor is between 2.5 cm and 5 cm and is limited to the lacrimal gland.
T3: The tumor has invaded the periosteum (the membrane of connective tissue that covers the bone around the eye).
T3a: The tumor is not larger than 5 cm.
T3b: The tumor is larger than 5 cm.
T4: The tumor has invaded the orbital soft tissues, optic nerve, or globe with or without extending to the bone; the tumor has extended beyond the orbit to adjacent structures, including the brain.
Node. The "N" in the TNM staging system stands for lymph nodes, the tiny, bean-shaped organs that help fight infection. Lymph nodes near the lacrimal gland are called regional lymph nodes. Lymph nodes in other parts of the body are called distant lymph nodes.
NX: The regional lymph nodes cannot be assessed.
N0 (N plus zero): There is no regional lymph node metastasis.
N1: There is regional lymph node metastasis.
Distant metastasis. The "M" in the TNM system indicates whether the cancer has spread from the lacrimal glands to other parts of the body.
MX: Distant metastasis cannot be assessed.
M0 (M plus zero): There is no distant metastasis.
M1: There is metastasis to other parts of the body.
Histologic grade. Histologic grade describes how closely the cancer cells resemble normal tissue under a microscope. A tumor's grade is described using the letter G and a number.
GX: The tumor grade cannot be identified.
G1: Describes cells that look more like normal tissue cells (well differentiated).
G2: The cells are somewhat different (moderately differentiated).
G3: The tumor cells look very much alike (poorly differentiated).
G4: The cells barely resemble normal cells (undifferentiated).
Used with permission of the American Joint Committee on Cancer (AJCC), Chicago, Illinois. The original source for this material is the AJCC Cancer Staging Manual, Sixth Edition (2002) published by Springer-Verlag New York,www.springer-ny.com.
The treatment of a lacrimal gland tumor depends on the size and location of the tumor, whether one or both eyes are involved, whether the cancer has spread, and the person's overall health. In many cases, a team of doctors may work with the patient to determine the best treatment plan.
Surgery
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 even the entire eye (enucleation), depending on the size and spread of the tumor.
The potential 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 sensation and pain impulses along the nerve pathways to the brain), and pain.
Radiation therapy
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. There are different types of radiation therapy. The dosage used, and the site and type of the tumor significantly affects the risks of complications.
External-beam radiation therapy. External-beam radiation therapy delivers x-rays from outside the body to the tumor and may be given as primary treatment, after surgical biopsy, or as palliative treatment (care given to improve quality of life by treating symptoms and side effects of the cancer or its treatment).
Proton beam or charged particle radiation therapy. This type of therapy targets high-energy particles (not x-rays) at the tumor. This type of radiation therapy can reduce the possibility of damage to nearby tissue.
Intensity modulated radiation therapy (IMRT). IMRT is a more advanced 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.
Radiation therapy may result in a variety of short-term and long-term side effects, so it is important to talk with your ophthalmologist and/or cancer doctor about what to expect. The extent of side effects depends on the type and dose of radiation therapy the person receives, where the tumor is located, and the person's general health.
Cataracts are a very common side effect of radiation therapy. 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. 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.
Chemotherapy
Chemotherapy is the use of drugs to kill cancer cells. Systemic chemotherapy is delivered through the bloodstream, targeting cancer cells throughout the body. The side effects of chemotherapy depend on the individual and the dose used, but 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 you've been prescribed, their purpose, and their potential side effects or interactions with other medications. Learn more about your prescriptions through Cancer.Net's Drug Information Resources, which provides links to searchable drug databases.
Immunotherapy
Immunotherapy is the use of substances (made by the body or created in a laboratory) to support or stimulate the body's own immune system to fight the cancer. Immunotherapy is also called biologic therapy. Examples of immunotherapy include cancer vaccines, monoclonal antibodies, and interferon.
Rituximab (Rituxan) is the most common immunotherapy used in the treatment of a lacrimal gland tumor and is used for the treatment of non-Hodgkin lymphoma.
Treatment by type of lacrimal gland tumor
Lymphoma
Lymphoma may be treated with external-beam radiation therapy, chemotherapy, immunotherapy, or a combination of these therapies. 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.
Benign mixed epithelial tumor
The most common form of treatment for a benign mixed epithelial tumor is an excisional biopsy (see Diagnosis). The chance of recovering from a benign mixed epithelial tumor of the lacrimal gland 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 form of treatment for AdCC is a procedure called exenteration. This procedure removes the lacrimal gland, eyeball, muscles, and all orbital contents and adjacent bone. A combination of chemotherapy and radiation therapy may also be used. Treatment is most successful when the cancer has not spread.
Doctors and scientists are always looking for better ways to treat patients with a lacrimal gland tumor. A clinical trial is a way to test a new treatment in order to prove that it is safe, effective, and possibly better than a standard treatment. Patients who participate in clinical trials are 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.
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 finding new drugs and other therapies is 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.
To join a clinical trial, patients must complete a learning process known as informed consent. During informed consent, the doctor should list all of the patient's options, so 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. Learn more about clinical trials, including patient safety, phases of a clinical trial, deciding to participate in a clinical trial, questions to ask the research team, and links to find cancer clinical trials.
The tumor and its treatment can cause a variety of side effects; some are easily controlled and others require specialized care. Below are some of the side effects that are more common to a lacrimal gland tumor and its treatments. For more detailed information on managing these and other side effects, visit Cancer.Net: Managing Side Effects.
Infection. An infection occurs when harmful bacteria, viruses, or fungi (such as yeast) invade the body and the immune system is not able to destroy them quickly enough. Patients with cancer are more likely to develop infections because both cancer and cancer treatments (particularly chemotherapy and radiation therapy to the bones or extensive areas of the body) can weaken the immune system. Symptoms of infection include fever (temperature of 100.5°F or higher); chills or sweating; sore throat or sores in the mouth; abdominal pain; pain or burning when urinating or frequent urination; diarrhea or sores around the anus; cough or breathlessness; redness, swelling, or pain, particularly around a cut or wound; and unusual vaginal discharge or itching.
Pain. Depending on the stage of disease, 30% to 75% of all patients experience pain from cancer. About 85% to 95% of cancer pain can be treated successfully. Pain can make other aspects of cancer seem worse, such as fatigue (tiredness), weakness, sleep disturbance, and confusion. Pain can come from the tumor itself or may be a result of cancer treatment. Pain from a tumor can be a result of the tumor growing and spreading to the bones or other organs and putting pressure on and damaging nerves. Pain from surgery is normal and may persist for months or years. Common procedures that cause pain afterward include mastectomy (removal of the breast and, occasionally, the surrounding tissue), chest surgery, neck surgery, and amputation of a limb (stump pain). Phantom pain is perceived pain in an organ or limb that has been removed. Pain may develop after radiation therapy and go away on its own. It can also develop months or years after treatment, especially after radiation therapy to the chest, breast, or spinal cord. Certain chemotherapeutic drugs can cause pain along with numbness in the fingers and toes. Usually this pain goes away when treatment is finished, but sometimes the damage can be permanent.
After treatment for 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.
Research involving more advanced diagnostic procedures and treatment for lacrimal gland tumors is ongoing. The following advancements may still be under investigation in clinical trials and may not be approved or available at this current time. Always discuss all diagnostic and treatment options with your doctor.
Radioimmunotherapy. Radioimmunotherapy is a treatment that combines the effectiveness of radiation treatment with immunotherapy. A radioactive material is attached to a protein that identifies the cancer 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 under investigation is ibritumomab tiuxetan (Zevalin), which is used in the treatment of lymphoma.
Radiation therapy. Proton-beam radiation therapy is being studied for its use in the treatment of a tumor around the eye.
Chemotherapy. Investigations continue to explore new chemotherapeutic drugs and combinations that may benefit people with a lacrimal gland tumor.
Regular communication with your doctor is important in making informed decisions about your health care. Consider asking the following questions of your doctor:
General questions:
What is my diagnosis?
What type of tumor is it? Is it benign or malignant?
How often do you treat people with this type of tumor?
If benign, will it turn malignant?
Do I need treatment right away?
What are my treatment options?
What treatment do you recommend?
What is the goal of this treatment?
What clinical trials are open to me?
Should I get a second opinion?
What is my prognosis?
What are the advantages of combining surgery and chemotherapy and radiation therapy?
What cosmetic issues will I face with treatment?
Will I need to see a plastic surgeon?
Will I need to see an ocularist (a person who makes prosthetic eyes) or an anaplastologist (a person who works with the surgery team to reconstruct the face)
For people who need surgery:
What side effects are possible from this type of surgery?
Can you recommend an experienced surgeon?
Will I need to stay in the hospital for this surgery? For how long?
How will you and I be able to tell if the entire tumor was removed during surgery?
For people who need radiation therapy:
What kind of radiation therapy will I receive?
What does the preparation for this treatment involve?
What other short-term and long-term side effects can I expect from this treatment?
How can you help relieve these side effects?
After treatment:
What are the chances the cancer will return?
What follow-up tests do I need, and how often do I need them?
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