Lymphoepithelioma-like carcinomacancer arising from tissues that line organs (LELC) is a rare type of carcinoma that resembles lymphoepithelioma, also known as undifferentiated nasopharyngeal carcinoma (NPC). For more information on nasopharyngeal cancers, please refer to the Rare Cancers Australia Nasopharyngeal cancer page.
LELC is most commonly found in the lungs (as a variant of non-small cell lung cancera disease where abnormal cells split without control and spread to other nearby body tissue and/or organs) and the skin (most commonly in the head and neck region), however it has also been found in the gastrointestinal tract, genitourinary tracts, and ocular adnexa (tissues surrounding the eye including the eyelids, conjunctival sac, and lacrimal gland).
LELC is generally diagnosed equally among the sexes, and is most commonly diagnosed in patients over the age of 50. However, anyone can develop this disease.
Treatment
If LELC 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 cellsthe basic structural and functional unit of all living things 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 (tumoura tissue mass that forms from groups of unhealthy cells) 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 tissuea group of cells that work together to perform a function.
- 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 LELC may include:
- Surgerytreatment involving removal of cancerous tissue and/or tumours and a margin of healthy tissue around it to reduce recurrence to remove as much of the tumour as possible (will vary based on location).
- 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.
- Targeted therapymedication that targets specific molecular features of cancer 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
While the cause of LELC remains unknown, the following factors may increase the likelihood of developing the disease:
- Infectiona condition where harmful pathogens, such as bacteria, viruses or parasites, have entered the body with the Epstein-Barr virus (EBV).
- Being immunocompromised.
- Being of Asian descent.
Not everyone with these riskthe possibility that something bad will happen factors will develop the disease, and some people who have the disease may have none of these risk factors. See your general practitioner (GP) if you are concerned.
Symptoms
The symptoms of LELC often vary with location.
General LELC Symptoms
General LELC symptoms may include:
- Persistent fevers.
- Excessive night sweats.
- Unexplained weight loss/loss of appetite.
- Fatiguea state of extreme tiredness or exhaustion, can be physical or mental.
- Headaches.
- Hypotensionlow blood pressure, which presents with its own symptoms:
- Light-headedness.
- Dizziness.
- Weakness.
- Blurred vision.
- Fatigue.
- Syncopefainting or passing out.
LELC of the Lungs
In addition to the general symptoms listed above, LELC of the lungs may cause:
- Persistent cough.
- Coughing up bloodthe red bodily fluid that transports oxygen and other nutrients around the body.
- Dyspneadifficulty breathing, shortness of breath.
- Chest pain.
- Heaviness in the chest.
- Changes in voice, such as hoarseness or loss of voice.
LELC of the Head and Neck Region
In addition to the general symptoms listed above, LELC of the head and neck region may cause:
- Palpable massa growth of cells that come together to make a lump, may or may not be cancer on the skin.
- Pain and/or redness in the eye.
- Watery eyes.
- Diplopiaseeing two images of a single object, also known as double vision.
- Proptosisbulging or protrusion of one or both of the eyes out of the eye sockets, also known as exophthalmos.
- Persistent runny nose.
- Unusual nosebleeds.
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 an LELC, 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:
- Chest x-raya type of medical imaging that uses x-ray beams to create detailed images of the body .
- 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.
- PET (positron emission tomography) scana type of medical imaging that uses radioactive tracers to create detailed images of the body.
- Ultrasounda type of medical imaging that uses soundwaves to create detailed images of the body .
- Blood teststesting done to measure the levels of certain substances in the blood.
- Pulmonary function testa test that examines how well your lungs are working.
- Sputum cytologyan examination of sputum (or mucus) from your lungs to see if any cancer cells are present.
- 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.