Lacrimal Gland Tumor

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What is Lacrimal Gland Tumor ?

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 multiply, they form a growth of tissue called a tumor. A lacrimal gland tumor can be benign (noncancerous) or malignant (cancerous, meaning it can spread to other parts of the body). There are four major types of lacrimal gland tumors:

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 not treated. This type of tumor begins in the cells that line the lacrimal gland.

Malignant mixed epithelial tumor. A malignant mixed epithelial tumor also begins in the cells that line the lacrimal gland. If it is not treated, 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 glands are the most common. Most ocular (eye-related) lymphoma is non-Hodgkin lymphoma, and may be associated with systemic (whole body) or central nervous system (brain and spinal cord) lymphoma.

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.

Symptoms and Signs

People with a lacrimal gland tumor may experience the following symptoms or signs. 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 or sign 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

Swelling around the eye

Double vision

Your doctor will ask you questions about the symptoms you are experiencing to help find out the cause of the problem, called a diagnosis. This may include how long you've been experiencing the symptom(s) and how often.

If cancer is diagnosed, relieving symptoms and side effects remains an important part of cancer care and treatment. This may also be called symptom management, palliative care, or supportive care. Be sure to talk with your health care team about symptoms you experience, including any new symptoms or a change in symptoms.

Risk Factors

A risk factor is anything that increases a person's chance of developing a tumor. Although risk factors often influence the development of a tumor, most do not directly cause a tumor. Some people with several risk factors never develop the disease, while others with no known risk factors do. However, knowing your risk factors and talking about them with your doctor may help you make more informed lifestyle and health care choices.

Age. A lacrimal gland tumor occurs more frequently in people in their 30s.

A 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 a previous benign tumor. In some instances, 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 evaluation after surgery is recommended for people having this type of surgery.

Diagnosis

Doctors use many tests to diagnose a tumor and find out if it has metastasized (spread). Some tests may also determine which treatments may be the most effective. For most types of tumors, 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

Type of tumor suspected

Severity of symptoms

Previous test results

In addition to a physical examination, the following tests may be used to diagnose a lacrimal gland tumor:

Biopsy. A biopsy is the removal of a small amount of tissue for examination under a microscope. Other tests can suggest that cancer is present, but only a biopsy can make a definite diagnosis. The sample removed from the biopsy is analyzed by a pathologist (a doctor who specializes in interpreting laboratory tests and evaluating cells, tissues, and organs to diagnose disease). The type of biopsy performed depends on the location of the tumor. 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. Sometimes, a contrast medium (a special dye) is injected into a patient's vein to provide better detail.

Magnetic resonance imaging (MRI). An MRI uses magnetic fields, not x-rays, to produce detailed images of the body. A contrast medium may be injected into a patient's vein to create a clearer picture.

Positron emission tomography (PET) scan. A PET scan is a way to create pictures of organs and tissues inside the body. A small amount of a radioactive substance is injected into a patient's body. This substance is absorbed mainly by organs and tissues that produce the most energy. Because cancer tends to use energy actively, it absorbs more of the radioactive substance. A scanner then detects this substance to produce images of the inside of the body.

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.

Stages and Grades

Staging is a way of describing where a tumor is located, whether it is cancerous, if or where it has spread, and whether it is affecting the functions of other organs in the body. Doctors use diagnostic tests to determine the tumor'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 tumors.

One tool that doctors use to describe the stage of a lacrimal gland tumor is the TNM system. This system judges three factors: 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 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 disease so doctors can work together to plan the best treatments. Not all doctors may use this staging system for lacrimal gland tumor; talk with your doctor for more information about staging.

TNM is an abbreviation for tumor (T), node (N), and metastasis (M). Doctors look at these three factors to determine the stage:

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 the tumor in even more detail. Specific tumor stage information is listed below:

TX: The primary tumor cannot be evaluated.

T0 (T plus zero): There is no tumor.

T1: The tumor is 2 centimeters (cm) or smaller and may or may not extend outside of the lacrimal gland to the orbital soft tissue.

T2: The tumor is between 2 cm and 4 cm and likely extends to the orbital soft tissue.

T3: The tumor is greater than 4 cm and likely extends to the orbital soft tissue.

T4: The tumor has invaded the periosteum (the membrane of connective tissue that covers the bone) or the orbital bone.

T4a: The tumor has invaded the periosteum.

T4b: The tumor has invaded the orbital bone.

T4c: The tumor has extended beyond the orbit to adjacent structures, including the brain and sinuses.

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 evaluated.

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 evaluated.

M0 (M plus zero): There is no distant metastasis.

M1: There is metastasis to other parts of the body.

Histologic grade. In addition to the TNM system, the doctor may describe a tumor by its grade. Histologic grade describes how closely the tumor 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).

Recurrent: Recurrent cancer is cancer that comes back after treatment. If there is a recurrence, the cancer may need to be staged again (called re-staging) using the system above.

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