Lacrimal gland cancera disease where abnormal cells split without control and spread to other nearby body tissue and/or organs is a rare type of ocular cancer that develops in the lacrimal glands, which are the small, tear-shaped glands in each eye. The lacrimal glands are responsible for secreting liquid (also known as tears) to clean, protect and lubricate the eyes.
The eye is a sensory organ that allows us to see by reacting to light. Light passes through the cornea (the clear, front layer of the eye) and enters the eye through an opening called the pupil (the black centre of the eye). The amount of light received by the pupil is controlled by the iris (the coloured portion of the eye). This light is then passed through the lens (the clear, inner portion of the eye) and works together with the cornea to focus light onto the retina (a layer of tissuea group of cells that work together to perform a function at the back of the eye that is sensitive to light). The retina then activates photoreceptors (image forming cellsthe basic structural and functional unit of all living things) to convert the light to electrical impulses that travel to the brain via the optic nerve, which is then converted into an image. The lacrimal gland is located above the eyeball in an area known as the orbit, which is the bony cavity of the skull containing the eyeball and its associated structures.
Lacrimal gland cancers are generally found equally among the sexes, and tend to be diagnosed around the age of 40. However, anyone can develop this disease at any age.
Types of Lacrimal Gland Cancer
There are several different types of lacrimal gland cancers, which are categorised by the types of cells they develop from within the lacrimal gland.
Benign Lacrimal Gland Masses
Benignnot cancerous, can grow but will not spread to other body parts lacrimal gland masses are tumours that are not cancerous or malignantcancerous, may grow and spread to other areas of the body. These tumours are often slow growing, and are not likely to spread to other parts of the body. In rare cases, a benign tumoura tissue mass that forms from groups of unhealthy cells can become malignant if left untreated. The most common benign lacrimal gland tumours include:
- Lacrimal gland pleomorphicmany forms; cells that have different size, shape etc. adenomaa benign tumour that develops from the epithelial lining of glands in the body.
- Lacrimal gland oncocytoma.
- Benign reactive lymphoid hyperplasia.
- Dacryops.
- Dermoid cyst of the lacrimal gland.
- Haemangiomas.
Adenoid Cystic Carcinoma
Adenoid cystic carcinomas (ACCs) are the most common subtype of lacrimal gland cancers that develop from tissues that line the lacrimal glands. These tumours are often associated with perineural spreading, which is when the cancer grows onto a nerve and uses it to travel and spread around the body. Lacrimal gland ACCs are often highly aggressive, and may not have as good of a prognosisto predict how a disease/condition may progress and what the outcome might be as other types of lacrimal gland cancers.
Adenocarcinoma
Adenocarcinomas are a very rare subtype of lacrimal gland tumours that develop from mucus-producing cells within the lacrimal gland. Because of how rare these tumours are, there has been limited research done into the aggressiveness and prognosis of this disease.
Acinic Cell Carcinoma
Acinic cell carcinomas are a very rare subtype of lacrimal gland cancer that develops from acinar cells, which are cells that assist in the secretion of liquid from the lacrimal glands. Because of how rare these tumours are, there has been limited research done into the aggressiveness and prognosis of this disease.
Leukaemia
Leukaemiacancer of blood and/or blood forming tissues is a very rare subtype of lacrimal gland cancer that develops from the bloodthe red bodily fluid that transports oxygen and other nutrients around the body or blood forming tissue within the lacrimal gland. Because of how rare these tumours are, there has been limited research done into the aggressiveness and prognosis of this disease.
The aggressiveness and prognosis of lacrimal gland leukaemia often varies by subtype. For more information on leukaemia, please refer to the Rare Cancers Australia Knowledgebase.
Lymphoma
Lymphomacancers of the lymphatic system is a very rare subtype of lacrimal gland cancer that develops from white blood cells in the lymphatic (immune) system. Unlike most other subtypes of lacrimal gland cancer, this disease is more common in females, and tends to be diagnosed in people between the ages of 50-70.
The aggressiveness and prognosis of lacrimal gland lymphoma often varies by subtype. For more information on lymphoma, please refer to the Rare Cancers Australia Knowledgebase.
Mucoepidermoid Carcinoma
Mucoepidermoid carcinomas (MECs) are a rare form of lacrimal gland cancer that develops from mucoepidermoid cells, which are mucus-secreting cells that line the lacrimal gland. Unlike most other subtypes of lacrimal gland cancer, this disease is more common in females, and tends to be diagnosed in people over the age of 60. MECs of the lacrimal gland can be aggressive, but can have a good prognosis when found early.
Solitary Fibrous Tumour
Solitary fibrous tumours (SFTs), also known as hemangiopericytomas, are a rare type of lacrimal gland tumour that generally develops in mesenchymal or fibrous tissue cells of the lacrimal glands. Mesenchymal tissue cells are those that help give organs shape and strength, and can develop into connective tissue, blood vessels and lymphatic tissue. Fibrous tissue cells are responsible for supporting, protecting, and holding bones, muscles, and other tissues and organs in place.
For more information on solitary fibrous tumours, please refer to the Rare Cancers Australia Solitary Fibrous Tumour page.
Squamous Cell Carcinoma
Squamous cell carcinomas (SCCs) are a common subtype of lacrimal gland cancer that develops from squamous cells, which are responsible for the filtration and diffusion of substances in the lacrimal gland. SCCs of the lacrimal gland can be aggressive, and can have a good prognosis when found early.
Treatment
If lacrimal gland cancer is detected, it will be staged and graded based on size, metastasiswhen the cancer has spread to other parts of the body, also known as mets, and how the cancer cells look under the microscope. Stagingthe process of determining how big the cancer is, where it started and if it has spread to other areas and grading helps your doctors determine the best treatment for you.
Cancers can be staged using the TNM staging system:
- T (tumour) indicates the size and depth of the tumour.
- N (nodea small lump or mass of tissue in your body) indicates whether the cancer has spread to nearby lymph nodessmall bean-shaped structures that filters harmful substances from lymph fluid.
- M (metastasis) indicates whether the cancer has spread to other parts of the body.
This system can also be used in combination with a numerical value, from stage 0-IV:
- Stage 0: this stage describes cancer cells in the place of origin (or ‘in situ’) that have not spread to nearby tissue.
- Stage I: cancer cells have begun to spread to nearby tissue. It is not deeply embedded into nearby tissue and had not spread to lymph nodes. This stage is also known as early-stage cancer.
- Stage II: cancer cells have grown deeper into nearby tissue. Lymph nodes may or may not be affected. This is also known as localisedaffecting only one area of body cancer.
- Stage III: the cancer has become larger and has grown deeper into nearby tissue. Lymph nodes are generally affected at this stage. This is also known as localised cancer.
- Stage IV: the cancer has spread to other tissues and organs in the body. This is also known as advancedat a late stage, far along or metastatic cancer.
Cancers can also be graded based on the rate of growth and how likely they are to spread:
- Gradea description of how abnormal cancer cells and tissue look under a microscope when compared to healthy cells I: cancer cells present as slightly abnormal and are usually slow growing. This is also known as a low-grade tumour.
- Grade II: cancer cells present as abnormal and grow faster than grade-I tumours. This is also known as an intermediate-grade tumour.
- Grade III: cancer cells present as very abnormal and grow quickly. This is also known as a high-grade tumour.
Once your tumour has been staged and graded, your doctor may recommend genetic testinga procedure that analyses DNA to identify changes in genes, chromosomes and proteins, which can be used to analyse tumour DNA to help determine which treatment has the greatest chance of success, which analyses your tumour DNA and can help determine which treatment has the greatest chance of success. They will then discuss the most appropriate treatment option for you.
Treatment is dependent on several factors, including location, stage of disease and overall health.
Treatment options for lacrimal gland cancers may include:
- Surgerytreatment involving removal of cancerous tissue and/or tumours and a margin of healthy tissue around it to reduce recurrence, potentially including:
- Resectionsurgical removal of tissue or part/all of an organ of the lacrimal gland.
- Orbital exenterationcomplete removal of the eyesocket, including the eyeball and surrounding tissues (muscles, nerves, fatty tissue, and potentially the eyelids).
- Radiation therapya treatment that uses controlled doses of radiation to damage or kill cancer cells.
- Chemotherapya cancer treatment that uses drugs to kill or slow the growth of cancer cells, while minimising damage to healthy cells.
- Clinical trialsresearch studies performed to test new treatments, tests or procedures and evaluate their effectiveness on various diseases.
- Palliative carea variety of practices and exercises used to provide pain relief and improve quality of life without curing the disease.
Risk factors
Because of how rare lacrimal gland cancers are, there has been limited research done into the riskthe possibility that something bad will happen factors of the disease.
Symptoms
The symptoms of lacrimal gland cancer may include:
- Pain and/or redness in the eye.
- Swelling of the eye(s).
- Proptosisbulging or protrusion of one or both of the eyes out of the eye sockets, also known as exophthalmos.
- A lump in or around the lacrimal gland.
- Changes in eyesight, potentially including:
- Diplopiaseeing two images of a single object, also known as double vision.
- Blurred vision.
- Watery eyes.
- Elevated intraocular pressure.
Not everyone with the symptoms above will have cancer, but see your general practitioner (GP) if you are concerned.
Diagnosis
If your doctor suspects you have lacrimal gland cancer, they may order the following tests to confirm the diagnosisthe process of identifying a disease based on signs and symptoms, patient history and medical test results and refer you to a specialist for treatment:
- Physical examinationan examination of your current symptoms, affected area(s) and overall medical history.
- Imagingtests that create detailed images of areas inside the body tests, potentially including:
- MRI (magnetic resonance imaging)a type of medical imaging that uses radiowaves, a strong magnet and computer technology to create detailed images of the body.
- CT (computed tomography) scana type of medical imaging that uses x-rays and computer technology to create detailed images of the body.
- Blood teststesting done to measure the levels of certain substances in the blood.
- Eye tests, potentially including:
- Ophthalmoscopyan examination of the back of the eye (fundus) to visualise the retina, optic disc and/or blood vessels; also known as a fundoscopy.
- Fluorescein angiographyan eye test that involves using a special dye and camera to examine blood flow in the retina and choroid.
- Electroretinographyan eye test that measures small electrical signals given off by the eye when exposed to different types of light.
- Biopsyremoval of a section of tissue to analyse for cancer cells.