What are the other Names for this Condition? (Also known as/Synonyms)

• EMECL (Epithelial-Myoepithelial Carcinoma of Lung)
• Malignant Mixed Tumor of Lung Comprising Myoepithelial and Epithelial Cells
• Pulmonary Epithelial-Myoepithelial Carcinoma

What is Epithelial-Myoepithelial Carcinoma of Lung? (Definition/Background Information)

• Epithelial-Myoepithelial Carcinoma of the Lung (EMECL) is an infrequent type of lung cancer that affects both men and women. The cause of formation of the cancer is unknown and so are the risk factors
• It is a low-grade tumor that occurs in middle-aged and older adults. No association with smoking has been reported in individuals with this lung cancer type
• Epithelial-Myoepithelial Carcinoma of Lung can present with chest pain, breathing difficulties, and other general signs and symptoms, such weight loss and fatigue
• Epithelial-Myoepithelial Carcinoma of the Lung is usually treated with a combination of surgery, chemotherapy and radiation. The lung cancer sometimes, shows a late recurrence. However, the prognosis of the tumor is generally good

Who gets Epithelial-Myoepithelial Carcinoma of Lung? (Age and Sex Distribution)

• Epithelial-Myoepithelial Carcinoma of Lung is an uncommon lung cancer type, affecting both middle-aged and older adults in the age range of 30-70 years (mean age 50 years)
• It is infrequent in children and teenagers
• Both males and females are affected
• Current studies do not show any racial or ethnic predilection

What are the Risk Factors for Epithelial-Myoepithelial Carcinoma of Lung? (Predisposing Factors)

• There are no known risk factors for Epithelial-Myoepithelial Carcinoma of Lung
• An association of the tumor with smoking is not observed

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases ones chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Epithelial-Myoepithelial Carcinoma of Lung? (Etiology)

The exact cause of Epithelial-Myoepithelial Carcinoma of Lung is unknown. The development of this carcinoma is not associated with smoking, unlike most other types of lung cancer.

• Information on genetic changes and abnormalities are not currently available. Involvement of the KRAS, EGFR, or PTEN genes have not been recorded
• In general, it is known that cancers form when normal, healthy cells begin transforming into abnormal cells - these cancer cells grow and divide uncontrollably (and lose their ability to die), resulting in the formation of a mass or a tumor
• The transformation of normally healthy cells into cancerous cells may be the result of genetic mutations. Mutations allow the cancer cells to grow and multiply uncontrollably to form new cancer cells
• These tumors can invade nearby tissues and adjoining body organs, and even metastasize and spread to other regions of the body

What are the Signs and Symptoms of Epithelial-Myoepithelial Carcinoma of Lung?

Early symptoms of Epithelial-Myoepithelial Carcinoma of Lung are similar to symptoms seen in common benign conditions and therefore, it is difficult to diagnose the cancer, at an early stage. Signs and symptoms associated with EMECL include:

• Coughing
• Labored breathing; shortness of breath
• Hemoptysis: Blood in cough is the most common sign
• Changes in one’s voice
• Fever due to recurring pneumonia
• Sudden weight loss
• The individual is easily tired, resulting in fatigue even with minimal activity
• Pain in the chest, if the tumor is pressing against a nerve
• In case of metastasis, pain at metastatic sites, such as bone

The tumor is usually located centrally (endobronchial location) and presents as a well-defined solid mass.

How is Epithelial-Myoepithelial Carcinoma of Lung Diagnosed?

There are a variety of tests the physicians may use to detect, locate, and diagnose Epithelial-Myoepithelial Carcinoma of Lung and to examine, if it has potentially spread to other regions. The initial tests may involve the following:

• Physical examination: During a physical exam, a physician will check the individual’s overall health status, listen to their breathing, and check for possible fluid buildup in the lungs (auscultation)
• Detailed medical history evaluation

A surgical procedure called a biopsy (usually performed by a radiologist or a cardiothoracic surgeon), is the only test a physician uses in order to make a definitive diagnosis of EMECL.

However, if a physician is unable to perform a lung biopsy due to the risk of damaging vital organs, additional tests will be recommended, and these may include:

• Chest X-ray: Two-dimensional pictures using tiny amounts of radiation are taken, in order to detect any tumors or other medical issues associated with the lungs, such as pneumonia. Tumors inside the lung, including Epithelial-Myoepithelial Carcinoma of the Lung, which is usually on the periphery, can be detected using a chest X-ray; although sometimes, these tumors are too small to visualize
• Computerized tomography (CT) scan: Also known as CAT scan, this radiological procedure creates detailed three-dimensional images of structures inside the body. CT scans are not a tool of preference to detect EMECL; although, CT scans may be helpful in detecting recurrences, or if the cancer has metastasized to the surrounding lymph nodes of the lungs
• Positron emission tomography (PET): A PET scan is a nuclear medicine imaging technique that uses three-dimensional images to show how tissue and organs are functioning. A small amount of radioactive material may be injected into a vein, inhaled or swallowed. A PET scan is also helpful in detecting recurrences, or if any metastasis (to the surrounding lymph nodes of the lungs) has occurred
• Magnetic resonance imaging (MRI): An MRI scan uses magnetic fields that create high quality pictures of certain body parts, such as tissues, muscles, nerves, and bones. These high-quality images may indicate to a physician, if any tumor is present
• Sputum cytology: Sputum cytology is test that involves the collection of mucus (sputum), coughed-up by a patient. After the mucus is collected, a pathologist examines it in an anatomic pathology laboratory, if any cell abnormalities are present indicative of the tumor
• Bone scan: A bone scan is a nuclear imaging test that involves injecting a radioactive tracer into an individual’s vein. Bone scans are primarily used to detect, if the cancerous cells in EMECL, has metastasized to the bones and formed secondary tumors
• Bone marrow biopsy: Bone marrow is a soft tissue found within bones. Occasionally, with Epithelial-Myoepithelial Carcinoma of Lung, a bone marrow biopsy is used to detect blood abnormalities, or if a physician thinks that metastasis to the bone marrow may have occurred

A biopsy refers to a medical procedure that involves the removal of cells or tissues, which are then examined by a pathologist. Different biopsy procedures include:

• Bronchoscopy: During bronchoscopy, physicians’ use a special medical instrument called a bronchoscope, which is inserted through the nose and into the lungs to collect small tissue samples. These samples are then examined by a pathologist, after the tissues are processed, in an anatomic pathology laboratory
• Thoracentesis: During thoracentesis, physicians use a special medical device called a cannula, to remove fluid between the lungs and the chest wall. A pathologist then examines the extracted cells from the fluid, in an anatomic pathology laboratory to determine any signs of Epithelial-Myoepithelial Carcinoma of Lung
• Thoracoscopy: During thoracoscopy, a surgical scalpel is used to make very tiny incisions into the chest wall. A medical instrument called a thoracoscope is then inserted into the chest, in order to examine and remove tissue from the chest wall, which are then examined further
• Thoracotomy: Thoracotomy is a surgical invasive procedure with special medical instruments to open-up the chest. This allows a physician to remove tissue from the chest wall or the surrounding lymph nodes of the lungs. A pathologist will then examine these samples under a microscope after processing the tissue in a laboratory
• Mediastinoscopy: During mediastinoscopy, a surgical scalpel is used to make very tiny incisions above the breastbone. Then, a medical instrument called a mediastinoscope is inserted into the chest wall to examine and remove samples. These tissue samples are processed and then examined under a microscope for any abnormalities
• Fine needle aspiration biopsy (FNAB): During fine needle aspiration biopsy, a device called a cannula is used to extract tissue or fluid from the lungs, or surrounding lymph nodes. These are then examined in an anatomic pathology laboratory, in order to determine any signs of Epithelial-Myoepithelial Carcinoma of the Lung
• Autofluorescence bronchoscopy: It is a bronchoscopic procedure in which a bronchoscope is inserted through the nose and into the lungs and measure light from abnormal precancerous tissue. Samples are collected for further examination by a pathologist

A differential diagnosis with respect to other lung cancer types may be necessary prior to establishing a definite diagnosis, by excluding the following cancers:

• Pleomorphic adenoma
• Adenoid cystic carcinoma
• Mucoepidermoid carcinoma
• PEComatous tumors
• Renal clear cell carcinoma

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Epithelial-Myoepithelial Carcinoma of Lung?

Complications of Epithelial-Myoepithelial Carcinoma of Lung may include:

• Dyspnea (shortness of breath): Individuals with EMECL may have an increased risk of exhibiting signs of dyspnea, if the cancerous tumor expands to block important large or small airways of the chest or lung
• Hemoptysis (coughing-up blood): Individuals may cough-up blood, due to excessive amounts of blood in the airways
• Pain: Advanced stages of EMECL that has metastasized to the pleural cavity (lining surrounding the lungs), invaded the chest wall, or other areas of the body, may cause excessive pain and agony
• Pleural effusion (fluid in the chest): Excessive fluid accumulation, resulting from cancerous cells inside the lungs, or outside of the lungs
• EMECL that spreads to other parts of the body: There is an increased risk of the cancer metastasizing (or spreading) to other parts of the body, such as the adrenal gland, liver, bones, and to the other lung

How is Epithelial-Myoepithelial Carcinoma of Lung Treated?

As with most cancers, early detection is linked with better survival results. The treatment method used is dependent on the location of the tumor, its type, and the stage of cancer. For Epithelial-Myoepithelial Carcinoma of Lung, the most commonly used treatment is surgery. Surgery can be potentially curative, if the tumor is completely excised (lower stage tumors). However, some cases show recurrence many years later. Chemotherapy and radiation may also be used for treatment, if surgery is not a viable option, or if there is a suspicion of metastasis.

• Chemotherapy: Chemotherapy drugs are typically taken as pills, or injected directly into a vein. These drugs travel through the body to kill any cancer cells. But, they can also kill healthy cells, located within the gastrointestinal tract and in hair follicles. The side effects include vomiting, nausea, and hair loss
• Radiation therapy: Radiation therapy involves using x-rays to kill cancerous cells. High-powered radiation beams are directed to particular body regions to target cancer. In the process, healthy cells can also be damaged. Radiation therapy can be administered in the following ways:
• Either by a machine, placed outside the body (called external beam radiation)
• Or it may be administered internally, by a device positioned directly at, or close to the malignant tumor, inside the lungs (called brachytherapy)

Radiation therapy and chemotherapy can be used as a combination therapy. Combinational therapy increases the effects of both types of treatment. However, the side effects are cumulative. Studies have shown that combination therapy of radiation and chemotherapy is not particularly useful, in Epithelial-Myoepithelial Lung Tumors.

Surgery: Depending on the stage of EMECL, surgery might be a viable option. However, the location of the tumor in the periphery makes it commonly associated with metastasis. The surgical options include:

• Wedge resection: Wedge resection is a partial removal of the wedge-shape portion of the lung that contains cancerous cells (along with any surrounding healthy tissue)
• Segmentectomy: Segmentectomy is also the partial removal of the cancerous lung and any surrounding healthy tissue
• Lobectomy (pulmonary lobectomy): Lobectomy is the most common type of surgical procedure performed for Epithelial-Myoepithelial Carcinoma of Lung, to partially remove a portion of a lung
• Sleeve lobectomy: Sleeve lobectomy is also a surgical procedure to partially remove a portion of the lung and a part of the airway (bronchus)
• Pneumonectomy: It is the surgical removal of the entire lung

How can Epithelial-Myoepithelial Carcinoma of Lung be Prevented?

Currently, there is no known prevention method for Epithelial-Myoepithelial Carcinoma of Lung. Although there are no preventable measures for lung cancer, in general, various steps can be taken to help decrease the risk of its formation. These measures include:

• Complete smoking cessation and avoiding exposure to secondhand smoke: The risk for lung cancer decreases drastically following quitting or giving up smoking
• Physical activity: Individuals, who participate in a moderate amount of physical activity, may decrease their risk
• Adequate consumption of fruits and vegetables: A healthy diet, low in saturated fats and rich in many fruits and vegetables, may help decrease one’s risk for lung cancer
• Avoid exposure to certain materials and chemicals (including asbestos, arsenic, chromium, nickel, and tar): Individuals who work with such substances should follow proper usage principles and occupational safety instructions, since a prolonged exposure to harmful chemicals may increase the risk for lung cancer. This risk is multiplied in smokers who are exposed to these harmful chemicals
• Avoid exposure to radioactive gas: Radon, a radioactive gas, produced by the natural breakdown of uranium in soil and rocks, may develop to hazardous levels inside building spaces. Individuals exposed to excessive amounts of radon gas are vulnerable to lung cancer
• Limit alcohol consumption: Alcoholic beverages have been linked to increasing an individual’s risk for certain types of cancers, such as those affecting the lungs, mouth, throat, esophagus, breast, colon, and liver
• CT screening in high-risk groups can decrease the incidence of lung cancer, or at least help detect such cancers early. A low-dose CT scan is good to identify the tumors early. It is generally known that CT scans are about 3-4 times better than X-ray studies

What is the Prognosis of Epithelial-Myoepithelial Carcinoma of Lung? (Outcomes/Resolutions)

• The prognosis of Epithelial-Myoepithelial Carcinoma of the Lung is good at lower stage and the tumor can be completely removed with surgery
• Tumor stage is the best indicator of tumor outcome; lower stage tumors have better outcomes than higher stage tumors
• Most tumors are not aggressive and the prognosis with adequate treatment is generally good. Metastasis of the tumor to other sites are rarely reported

Epithelial-Myoepithelial Carcinoma of Lung is staged as follows:

• Stage I: Epithelial-myoepithelial carcinoma is only in the lung
• Stage II: Epithelial- myoepithelial carcinoma is in the lung and has metastasized to adjacent lymph nodes
• Stage IIIA: Epithelial- myoepithelial carcinoma is in the lung and the lymph nodes, on the same side of the chest, as the cancer
• Stage IIIB: Epithelial- myoepithelial carcinoma is in the lung and the lymph nodes, on the opposite side of the chest as the cancer, or is above the collar bone
• Stage IV: Epithelial- myoepithelial carcinoma has spread to other side of the lung and other parts of the body

Furthermore, as with other forms of cancer, early detection is correlated with higher survival rates. For patients diagnosed with Epithelial-Myoepithelial Carcinoma of Lung, it is important to have follow-up appointments with a physician, to evaluate the effects of the current treatment method, and to monitor for any returning tumors.

Additional and Relevant Useful Information for Epithelial-Myoepithelial Carcinoma of Lung:

Epithelial-Myoepithelial Carcinoma of the Lung causes spindle-like (connective tissue), clear or plasmocytoid (plasma cells) appearance of myoepithelial cells of the lung and epithelial cells, which form the ducts in the lung.