Pulmonary epithelioid haemangioendothelioma (pulmonary EHE) is a very rare type of cancer that develops from epithelial cells in the lining of the lung. Haemangioendothelioma is a term used to describe neoplasms that act in-between a hemangioma (a benignnot cancerous, can grow but will not spread to other body parts growth composed of blood vessel cells) and an angiosarcoma (a malignancy found in the lining of blood vessels).
The respiratory system is composed of the nose, mouth, trachea and airways (bronchi and bronchioles) that lead to the lungs, which is where the breathing process primarily occurs. Each lung is made up of lobes, with the right lung being composed of three lobes and the left only having two lobes to accommodate room for the heart. The lungs sit on top of a muscle called the diaphragm, which separates the abdomen from the chest. The diaphragm works by contracting/flattening when we inhale, pulling air into the lungs. When we exhale, the diaphragm relaxes and pushes air out of the lungs. The space between the lungs is called the mediastinum, and holds several important structures, including the heart, trachea, oesophagus and lymph nodessmall bean-shaped structures that filters harmful substances from lymph fluid. Each lung is covered by a layer of tissue called the pleura.
Pulmonary EHE is more commonly diagnosed in females, and tends to be diagnosed between the ages of 25-54. However, anyone can develop this disease.
Treatment
When cancers are detected, they are staged and graded based on size, metastasis, and how the cancer cells look under the microscope. Staging and grading helps your doctors determine the best treatment for you. However, because of how rare pulmonary EHEs are, there is currently no standard staging and grading system for this disease. Instead of staging and grading, your doctor will recommend a treatment plan based on the following factors:
- Cancer location.
- Whether or not the cancer has metastasised.
- Your age.
- General health.
- Your treatment preferences.
Your doctor may also 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 options for pulmonary EHE may include:
- Surgery to remove as much of the tumour as possible.
- Chemotherapya cancer treatment that uses drugs to kill or slow the growth of cancer cells, while minimising damage to healthy cells.
- Radiation therapya treatment that uses controlled doses of radiation to damage or kill 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
Because of how rare pulmonary EHEs are, there has been limited research done into the risk factors of this disease.
Symptoms
Symptoms of pulmonary EHE may include:
- Chest tightness.
- Dyspnea.
- Persistent cough.
- Haemoptysiscoughing up blood.
- Fatigue.
- Pleural effusionabnormal fluid build-up in the pleural space, which can cause chest pain and breathing difficulties (fluid in the chest).
Not everyone with the symptoms above will have cancer, but see your general practitioner (GP) if you are concerned.
Diagnosis
In some cases, pulmonary EHE can be found incidentally while investigating another condition. If your doctor suspects you have pulmonary EHE, they may order the following tests to confirm the diagnosis and refer you to a specialist for treatment:
- Physical examinationan examination of your current symptoms, affected area(s) and overall medical history.
- Blood teststesting done to measure the levels of certain substances in the blood.
- Imaging tests, potentially including:
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- 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
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- CT (computed tomography) scana type of medical imaging that uses x-rays and computer technology to create detailed images of the body.
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- Chest X-raya type of medical imaging that uses x-ray beams to create detailed images of the body .
- 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.
- Thoracentesisa procedure that drains the fluid from your lungs using a needle.
- Exploratory surgerya surgical procedure used for conditions that cannot be confirmed by scans and tests alone, potentially including:
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- Bronchoscopyan examination of the trachea and lungs with a small, flexible instrument known as a bronchoscope.
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- Mediastinoscopyexamination of the mediastinum (area between the lungs) with a small, flexible instrument called a mediastinoscope .
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- Thoracoscopyexamination of the inside of the chest cavity with a small, flexible instrument known as a thorascope.
- Biopsyremoval of a section of tissue to analyse for cancer cells.