Types of Treatment for Lacrimal Gland Tumor

Executive Summary

Treatment (lacrimal gland tumor) recommendations depend upon the size, grade and type of tumor, metastasis, possible side effects, and patient’s preferences and overall health. The standard treatment techniques used to treat lacrimal gland tumors are Surgery, removal of the eye, radiation therapy (Proton therapy, External-beam radiation therapy, Intensity-modulated radiation therapy (IMRT)), chemotherapy, and immunotherapy. There are treatment strategies which are used to treat specific types and stages of lacrimal gland tumors. The preferred treatment methods used to treat lymphoma are chemotherapy, external-beam radiation therapy, immunotherapy, or a combination of these treatments. An excisional biopsy is the preferred treatment procedure for treating a benign mixed epithelial tumor. Complete surgical removal is the ideal treatment strategy to treat a malignant mixed epithelial lacrimal gland tumour. The standard type of treatment strategy preferred is a procedure called exenteration for ACC of lacrimal gland tumour. Palliative care includes medication, nutritional changes, emotional and spiritual support and other relaxation therapies. The treatment procedures for recurrent tumors include Surgery, radiation therapy etc. Many clinical trials are testing the viability of various treatment options for dealing with recurrent lacrimal gland tumors.

Treatment for Lacrimal Gland Tumor

For treating a condition like lacrimal gland tumors, doctors from different areas of expertise work together to design a suitable treatment plan. There will be a multidisciplinary team consisting of different doctors to carry out the treatment. The team will consist of various healthcare professionals, including oncologists, physician assistants, oncology nurses, nurse practitioners, pharmacists, dieticians, counsellors, and social workers. Also, patients who received radiation treatment for the eye may need to consult a plastic surgeon or ophthalmologist to ensure that their eyes are still functional after treatment. In addition to treatment, the healthcare team will also focus on providing care and support to the family members associated with the patient.

Patients should have the impression that their doctors are working together on a coordinated plan of care and are communicating effectively. Patients should talk with their doctors or seek additional opinions before treatment if they believe that the team is not communicating properly with them or on the treatment goals and plan of care.

The following are descriptions of the most frequent treatment methods used to treat lacrimal gland tumors. The treatment’s goals include removing the tumor entirely and maintaining the patient’s eye’s health and eyesight.

Doctors design the treatment plan based on several factors, like:

  • The type of tumour
  • Stage and grade of the tumor
  • Probable side effects
  • Whether the tumour has affected one or both eyes.
  • Whether the tumour is benign or malignant (cancerous)
  • Patient’s choices and preferences
  • The overall health of the patient.

The treatment plan will also focus on managing and treating the associated symptoms and side effects of the disease ​1​. Patients should take time to learn about all the available treatment options for lacrimal gland tumors and be sure to ask questions and clear doubts about things that are unclear and appears complex. Ask your doctor about the aim of each treatment and what outcome you can expect while receiving the treatment. This is what is called “shared decision making.” Shared decision making helps the patients to have informed choices regarding treatment. This will help the healthcare team choose and plan a treatment strategy that best fits your aims and expectations.

SURGERY

A Surgery is a procedure where the tumor and some surrounding healthy tissues are removed via an operation. A lacrimal gland tumor is commonly treated with surgery on the eye. The ophthalmologist will remove components of the damaged eye during the procedure. Depending on the size and extent of the tumor spread, the entire eye (called enucleation) may be removed.

Eye surgery might have the same adverse effects as any other surgical procedure. Risk of infection, anaesthesia (medication used before surgery to decrease pain perception), and pain are some of them.

Removing the eye(s)

Sometimes it is essential to remove the eye for medical reasons. A person with one eye may have difficulty with depth perception due to this vision loss. The majority of people adjust to these variations with the help of their health care team.

Most people are concerned about appearing after having an eye removed. Today’s cosmetic surgery produces good results in the majority of cases. The patient is fitted for a prosthesis, an artificial eye, to fill the gap left by the missing eye. The prosthesis will resemble a normal eye in appearance and behavior. The prosthetic eye, for example, will move in tandem with the person’s remaining eye, but not as much as a real eye does. Family members may detect whether the eye is fake, but visitors are unlikely to notice.

If enucleation is required, discuss the possibility of a prosthetic with your doctor. It could take many weeks for you to get one. Also, inquire about any accessible support programmes to assist you in adjusting to the loss of an eye.

RADIATION THERAPY

Radiation therapy refers to the use of high energy x-rays or other particles to destroy tumor growth. A radiation oncologist is a doctor who specializes in performing this procedure. A radiation therapy schedule or regimen usually consists of several treatments given over a set period.

Types of radiation therapy procedures used to treat lacrimal gland tumors are as follows ​2​:

  • Proton therapy is a type of external beam radiation procedure that uses protons instead of x-rays to eliminate the tumor cells. The procedure is also called proton-beam therapy.
  • External-beam radiation therapy: the most common type of radiation therapy, where external-beam radiation is given from a machine outside the body. 
  • Intensity-modulated radiation therapy (IMRT): IMRT is another way to carry out external-beam radiation therapy. The intensity of the radiation is adjusted to precisely target the tumor growth. Hence, the procedure causes significantly less damage to the surrounding healthy tissue when compared to the conventional radiation procedure. The process also causes very little damage to the nearby vital organs.

Radiation procedures can cause various side effects. The type of the tumour, location and dosage of radiation used determine the associated side effects and risks. Common side effects of radiation procedures in and around the eye include: 

Cataracts: When the eye lens becomes hazy, it is called a cataract. Cataract patients may experience blurry or foggy vision, difficulty seeing at night, and/or glare from the sun or bright lights. A cataract can be surgically removed if it creates significant problems with a person’s vision.

Loss of eyelashes and/or a dry eye: some people may experience a dry eye or loss of eyelashes, especially if they have undergone external-beam radiation therapy and proton-beam radiation therapy.

Some other less common side effects of radiation therapy are:

  • Radiation optic neuropathy: refers to damage to the optic nerve.
  • Radiation retinopathy: refers to the formation of abnormal blood vessels in the retina.
  • Neovascular glaucoma: refers to a painful condition where new blood vessels form and prevent fluid outflow from the eyes.

If radiation therapy causes considerable damage to the eye, the eye may need to be removed. Fatigue, moderate skin responses, unsettled stomach, and loose bowel motions are other adverse effects of radiation therapy. The majority of these side effects subside once treatment is completed. Discuss the risks and advantages of various types of radiation therapy with your doctor.

CHEMOTHERAPY

Chemotherapy or chemo procedure refers to drugs or medications to eliminate cancer cells. These drugs operate by preventing the cancer cells from growing, dividing and generating more cells. But normal cells also grow and divide, and chemo procedure can affect these cells, causing a range of side effects.

A chemo regimen generally consists of several cycles given over some time. A patient may receive a single type of drug or a combination of different medications, all given simultaneously. 

A medical oncologist gives chemo medications. The medication is provided either via an intravenous (IV) tube placed in the vein or in the form of a capsule or pill to be swallowed. For treating a lacrimal gland tumour, sometimes the chemo medication is given via the blood vessel (intra-arterial) by inserting a catheter through the groin area, such that the medication reaches the eye and the tumour. The doctor may use imaging technology to guide the catheter to reach the blood vessel in the head.

Chemotherapy can cause many side effects. These side effects depend on the type of drug and the dose used. Some side effects include nausea and vomiting, fatigue, hair loss, risk of infection, loss of appetite, and diarrhoea. These side effects usually go away once the treatment finishes. In some rare cases, chemotherapy can cause long-term problems that can affect the kidneys’ heart and even cause cancer. Please talk with your doctor about the medication used to treat the tumor condition, its purpose and potential side effects.

IMMUNOTHERAPY

Immunotherapy is also called biologic therapy. The medicated treatment focuses on improving the body’s natural defences to fight and destroy cancer growth. This therapy uses materials made either naturally by the body or in a lab to improve, target or restore the functions of the immune system.

The most common immunotherapy drug used to treat lacrimal gland tumors is Rituximab (Rituxan). The medication is also used to treat non-Hodgkin lymphoma.

TREATMENT BY LACRIMAL GLAND TUMOUR TYPE

Below are some treatment strategies used to treat specific types and stages of lacrimal gland tumors. Patients should openly talk with their doctor or healthcare team regarding all the available treatment options to treat their tumor condition. Patients are also encouraged to inquire about any clinical trials they can participate in as per their disease condition’s type and stage. 

  • Lymphoma: The preferred treatment methods used to treat lymphoma are chemotherapy, external-beam radiation therapy, immunotherapy, or a combination of these treatments ​3​. If only one eye is affected by the tumor, then recovery is very high. The treatment procedure for ocular lymphoma depends on whether the tumor has affected other body parts or not. Hence for treating this type of tumor, knowing the stage is extremely important.
  • Benign mixed epithelial tumor: An excisional biopsy is the preferred treatment procedure for treating a benign mixed epithelial tumor. In this, the tumor is removed surgically. The prognosis and recovery become even more smooth if the tumor is eliminated.
  • Malignant mixed epithelial tumor: Complete surgical removal is the ideal treatment strategy to treat a malignant mixed epithelial lacrimal gland tumor. 
  • ACC of the lacrimal gland: ACC is considered an aggressive form of cancer, and the standard type of treatment strategy preferred is a procedure called exenteration. The surgeon removes the lacrimal gland, eyeball, muscles, orbital contents, and adjacent bone in this treatment procedure. Sometimes a combination of radiation therapy and chemotherapy may also be used as part of the treatment plan. Treatment becomes more successful if the tumor has not metastasized to other parts or tissues of the body.

Physical, emotional, and social effects of a tumor

A tumor and its treatment can cause many physical, emotional and social effects. Tumor care doesn’t end with active treatment. Post-treatment care is as critical as active treatment. Palliative or supportive care focus on managing and relieving symptoms and side effects associated with cancer treatment. Palliative care is carried out along with an active treatment strategy, which helps in improving the treatment efficiency by slowing the cancer growth or destroying it.

Palliative care focuses on improving a patient’s condition by helping them to manage and relieve symptoms. The care extends support not just to the patients but also to their loved ones. It also focuses on the non-medical needs of cancer patients, like managing finances. Palliative care depends on the patient’s age, stage, or tumour type. Palliative care works best when it is begun right after a cancer diagnosis. This helps prevent the cancer condition and its symptoms and side effects from aggravating. Palliative focuses on improving the comfort and quality of a patient’s life. Palliative care enhances the effectiveness of active treatment. Sometimes a patient may receive chemotherapy, surgery or radiation therapy as palliative care.  

Patients should talk with their health care team about the aim of each treatment option, about the probable side effects and the available palliative care options before the active treatment begins. The health care team will enquire about your medical history, symptoms and side effects during treatment. Be sure to communicate whatever you are feeling to the doctor. This helps them treat any symptoms or side effects that you are experiencing with ease. This will eventually help prevent your condition from becoming worse. 

Metastatic lacrimal gland tumour

Metastatic cancer refers to a cancer or tumor that spreads from its origin to different body parts. If you are experiencing any signs or symptoms related to cancer metastasis, communicate it with your doctor. The doctor or the health care team will develop an active treatment plan to cure this condition. One can also go for clinical trial options. Metastasis can occur even after active treatment and removal of the cancer growth. Hence it can be stressful for the patient and their loved ones to bear the diagnosis. So, patients and their loved ones are encouraged to have active communication with the doctor, nurses, social workers or counsellors regarding how they are feeling. Patients can also talk with other cancer survivors or join a support group.

Remission and the chance of recurrence

Remission refers to a stage where there are no signs or symptoms of a tumor in the body. The condition can be called NED or “no evidence of disease” in the body. Remission can be permanent or temporary. Cancer patients in remission are constantly worried about a probable cancer recurrence. Patients must continue tests, scans, physical examinations even after successful treatment to look for recurrence. Patients with lacrimal gland tumors should talk with their doctor about the chance of the tumor condition recurring back in the body. If cancer recurs in the same place as before, it is called local recurrence, and if it happens nearby the origin point, it is called a regional recurrence. If it recurs in some distant place in the body, it is called distant recurrence.

The doctor will check for any signs of tumor recurrence. And if the tumor growth is back in the body, the doctor will list out the possible treatment options. In most cases, the treatment procedures for recurrent tumors include surgery, radiation therapy etc., but the same kind of radiation procedure cannot be given more than once. These therapies may be performed in different combinations.

Many clinical trials are testing the viability of various treatment options for dealing with recurrent lacrimal gland tumors. Patients are encouraged to talk with their doctor about any such possibilities. Whatever the treatment plan be, palliative care will always be an essential part that will primarily focus on managing and relieving symptoms and side effects of treatment.

When a tumor recurs back in the body or the disease worsens, it is quite natural and entirely ok for patients and their loved ones to feel upset and be in extreme fear. In such cases, both the patients and their caretakers are encouraged to talk to the healthcare team or counsellors or seek support services or groups to cope with the circumstances. 

What If the treatment does not work?

Tumor recovery may not be successful at times. The disease condition is advanced or terminal if it cannot be controlled or cured. Any stage of cancer or tumor can be stressful to people. A diagnosis that states an advanced, aggressive tumor can cause even more stress and make people hopeless and uncertain about their lives. Patients should have open, honest communications with their doctor or health care team about their concerns, feelings and preferences. Health care team members are skilled and will support the patients and their caregivers. They will focus on improving the comfort and quality of the patient’s life by providing them physical, mental and financial support.

People diagnosed with advanced tumors and the expected chance of survival is for less than six months can consider hospice care. Hospice care focuses on providing the best possible quality care for patients whose survival chance is low. Patients and their families can talk to the health care team about the available hospice options, including a unique hospice centre, hospice care at home or any other health care location. People can also avail nursing care options.

References

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    Woo KI, Kim YD, Sa HS, Esmaeli B. Current treatment of lacrimal gland carcinoma. Current Opinion in Ophthalmology. Published online September 2016:449-456. doi:10.1097/icu.0000000000000301
  2. 2.
    Brada M, Henk JM. Radiotherapy for lacrimal gland tumours. Radiotherapy and Oncology. Published online July 1987:175-183. doi:10.1016/s0167-8140(87)80228-1
  3. 3.
    Mohammad AENA, Kroosh SS. Treatment of primary lymphoma of the lacrimal gland by surgical excision alone: A 5-year follow-up study. Orbit. Published online January 2001:131-140. doi:10.1076/orbi.20.2.131.2639