Pulmonary epithelioid haemangioendothelioma (pulmonary EHE) is a very rare type of cancera disease where abnormal cells split without control and spread to other nearby body tissue and/or organs that develops from epithelial cellsthe basic structural and functional unit of all living things 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 bloodthe red bodily fluid that transports oxygen and other nutrients around the body vessela tube that carries bodily fluid, such as blood or lymph fluid, around the body cells) and an angiosarcoma (a malignancy found in the lining of blood vessels).
The respiratory system is composed of the nose, mouth, tracheathe tube that connects your voicebox (larynx) to the lungs, also known as a windpipe 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 abdomenstomach, stomach area, belly 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 mediastinumthe space between the lungs that holds many important structures, including the heart, trachea and oesophagus, 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 tissuea group of cells that work together to perform a function called the pleuraa thin layer of tissue covering the lungs and the wall of the interior chest cavity.
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, metastasiswhen the cancer has spread to other parts of the body, also known as mets, 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 stagingthe process of determining how big the cancer is, where it started and if it has spread to other areas 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:
- 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 tumoura tissue mass that forms from groups of unhealthy cells 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 riskthe possibility that something bad will happen factors of this disease.
Symptoms
Symptoms of pulmonary EHE may include:
- Chest tightness.
- Dyspneadifficulty breathing, shortness of breath.
- Persistent cough.
- Haemoptysiscoughing up blood.
- Fatiguea state of extreme tiredness or exhaustion, can be physical or mental.
- 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 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.
- Blood teststesting done to measure the levels of certain substances in the blood.
- Imagingtests that create detailed images of areas inside the body 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.