context: When cells of the lung start growing rapidly in an uncontrolled manner, the condition is called lung cancer. Cancer can affect any part of the lung, and it's the leading cause of cancer deaths in both women and men in the United States, Canada, and China. There are two main types of lung cancer. Small-cell lung cancer ( SCLC), sometimes called small-cell carcinoma, causes about 10%-15% of all lung cancer. Non-small-cell lung cancer ( NSCLC) causes the rest. There are 2 main types: Small-cell carcinoma (oat cell cancer) Combined small-cell carcinoma Both include many types of cells that grow and spread in different ways. They are named according to what the cells look like under a microscope. Small-cell lung cancer differs from non-small-cell lung cancer in the following ways: Small-cell lung cancer grows rapidly. Small-cell lung cancer spreads quickly. Small-cell lung cancer responds well to chemotherapy (using medications to kill cancer cells) and radiation therapy (using high-dose X-rays or other high-energy rays to kill cancer cells). Small-cell lung cancer is frequently associated with distinct paraneoplastic syndromes (a collection of symptoms that result from substances produced by the tumor). The predominant cause of both small-cell lung cancer and non-small-cell lung cancer is tobacco smoking. However, small-cell lung cancer is more strongly linked to smoking than non-small cell lung cancer. Even secondhand tobacco smoke is a risk factor for lung cancer. Those living with a smoker have an almost 30% increase in the risk of developing lung cancer compared to people who are not exposed to secondhand smoke. All types of lung cancer occur with increased frequency in people who mine uranium, but small-cell lung cancer is most common. The prevalence is increased further in persons who smoke. Exposure to radon (an inert gas that develops from the decay of uranium) has been reported to cause small-cell lung cancer. Exposure to asbestos greatly increases the risk of lung cancer. A combination of asbestos exposure and cigarette smoking increases the risk even further. Persons with small-cell lung cancer typically have had symptoms for a relatively short time (8 to 12 weeks) before they visit their doctor. The symptoms can result from local growth of the tumor, spread to nearby areas, distant spread, paraneoplastic syndromes, or a combination thereof. Symptoms due to local growth of the tumor include the following: Cough Coughing up blood Shortness of breath Chest pain worsened by deep breathing Symptoms due to spread of the cancer to nearby areas include the following: Hoarse voice, resulting from compression of the nerve that supplies the vocal cords Shortness of breath, resulting from compression of the nerve that supplies the muscles of the diaphragm, or the lungs filling with fluid and stridor (sound produced by turbulent flow of air through a narrowed part of the respiratory tract) resulting from compression of the trachea (windpipe) or larger bronchi (airways of the lung) Difficulty swallowing, resulting from compression of the esophagus (food pipe) Swelling of the face and hands, resulting from compression of the superior vena cava (vein that returns deoxygenated blood from the upper body) Symptoms due to distant cancer spread depend on the site of spread and can include the following: Spread to the brain can cause headache, blurring of vision, nausea, vomiting, weakness of any limb, mental changes, and seizures. Spread to the vertebral column can cause back pain. Spread to the spinal cord can cause paralysis and loss of bowel or bladder function. Spread to the bone can cause bone pain. Spread to the liver can cause pain in the right upper part of the abdomen. Symptoms due to paraneoplastic syndromes include the following: Symptoms may or may not be characteristic of a specific organ system. Nonspecific symptoms include fatigue, loss of appetite, and weight gain or loss. Severe muscle weakness. Trouble with balance or walking. Changes in mental status. Changes in skin color, texture, and facial features. Consult a doctor if any of the following symptoms are present: Shortness of breath Coughing up blood Unexplained weight loss Voice change New cough or change in the consistency of a cough Unexplained persistent fatigue Unexplained deep aches or pains Call 911 if any of the following symptoms are present: Coughing up large amounts of blood Chest pain Sudden shortness of breath Sudden or severe weakness of any limb Sudden vision problems Seizures Initial exams and tests for suspected lung cancer may include the following: Medical, surgical, work, and smoking history Physical exam to check for general signs of health Chest X-ray CT scan of the chest: An X-ray machine linked to a computer takes a series of detailed pictures of the inside of the chest from different angles. Other names of this procedure are computed tomography, computerized tomography, or computerized axial tomography. Thoracentesis: The lungs are enclosed in a sac. Lung cancer can cause fluid to collect in this sac. This is called pleural effusion. In people who have cancer, this fluid may contain cancer cells. The fluid is removed by a needle and examined for the presence of cancer cells. Bronchoscopy: This is a procedure used to look inside the trachea (windpipe) and large airways in the lung for abnormal areas. A bronchoscope (a thin, flexible, lighted tube with a tiny camera on the end) is inserted through the mouth or nose and down the windpipe. From there, it can be inserted into the airways (bronchi) of the lungs. During bronchoscopy, the doctor looks for tumors and takes a biopsy (a sample of cells that is removed for examination under a microscope) from the airways. Lung biopsy: If a tumor is on the periphery of the lung, it may not be seen with bronchoscopy. Instead, a biopsy sample has to be taken with the help of a needle inserted through the chest wall and into the tumor. This procedure is called a transthoracic needle biopsy. Mediastinoscopy: This procedure is performed to determine the extent the tumor has spread into the mediastinum (the area of the chest between the lungs). Mediastinoscopy is a procedure in which a tube is inserted behind the breastbone through a small cut at the lowest part of the neck. Samples of the lymph nodes (small, bean-shaped structures found throughout the body) are taken from this area to look for cancer cells. Once the patient has been diagnosed with lung cancer, exams and tests are performed to find out whether the cancer has spread (metastasized) to other organs. These tests help determine the stage of the cancer. Staging is important, because lung cancer treatment is based on the stage of the cancer. Tests used to detect the spread of cancer may include the following: Blood tests: Complete blood count -- CBC -- provides information about the type and count of different types of blood cells, serum electrolytes, kidney function, and liver function. In some cases, these tests may identify the site of metastasis. These tests are also important to assess the organ functions before starting treatment. CT scan of the chest and abdomen: An X-ray machine linked to a computer takes a series of detailed pictures of areas inside the body from different angles. The doctor may inject a dye into a vein. A contrast agent may be given to swallow so that the organs or tissues more clearly show up on the scan. MRI: MRI is an imaging technique used to produce high-quality images of the inside of the body. A series of detailed pictures of areas inside the body are taken from different angles. The difference between an MRI and CT scan is that MRI uses magnetic waves, whereas CT scan uses X-rays for the procedure. Radionuclide bone scan: With the help of this procedure, the doctor determines whether the lung cancer has spread to the bones. The doctor injects a minute quantity of radioactive material into the vein; this material travels through the bloodstream. If the cancer has spread to the bones, the radioactive material collects in the bones and is detected by a scanner. PET scan: A small amount of radioactive material is injected into the bloodstream and measures the metabolism of the organs to see if the cancer has spread. Video-assisted thoracoscopy ( VATS): A doctor will insert a lighted tube with a video camera through small openings in the chest. It's a way to look at the lungs and other tissue. A biopsy may also be done. Endobronchial ultrasound ( EBUS): A doctor inserts a flexible tube with a video camera and an ultrasound attached, through your mouth and into your windpipe and lungs. They can look at the lungs and lymph nodes nearby and can take a biopsy of the tissue. Staging Staging of the cancer provides important information about the outlook of the patient's condition and helps the doctor plan the best treatment. Although other cancers are categorized from stage I to stage IV, small-cell lung cancer is classified in two stages. Limited stage: In this stage, the tumor is confined to one side of the chest, the tissues between the lungs, and nearby lymph nodes only. Extensive stage: In this stage, cancer has spread from the lung to other parts of the body. Some of the most commonly used medications for the treatment of persons with small-cell lung cancer are cisplatin, etoposide, vincristine, doxorubicin, irinotecan, topotecan, paclitaxel, docetaxel and cyclophosphamide. Standard treatment of small-cell lung cancer involves combination chemotherapy with a cisplatin-containing regimen. Treatment cycles are typically repeated every three weeks. People receive treatment for four to six cycles. Radiotherapy to the chest may be started as early as possible, or it may be given later in the course of treatment. This depends on factors such as the stage of the cancer and the person's overall health. Radiation and chemotherapy: Sequential-radiation treatment may be given, followed by chemotherapy. However, in comparative studies, the earlier the radiation is started concurrently with chemotherapy (as early as the first cycle of chemotherapy), the better the outcome. If the patient has limited disease, and has had a very good response to chemotherapy, radiation therapy may be given to the patient's brain to reduce the risk of small-cell lung cancer spreading to the brain. This is called prophylactic cranial irradiation ( PCI). It is usually given after the patient has completed the full chemotherapy, and radiotherapy (to the thorax). The radiation doses are low, and the treatment duration is short, so the side effects of this therapy are minimal. Treatment of extensive-stage small-cell lung cancer (small-cell lung cancer that remains incurable with current treatment options) Persons with extensive-stage small-cell lung cancer are treated with combination chemotherapy. Currently, the combination of cisplatin or carboplatin and etoposide ( PE) is the most widely used regimen. Radiation therapy may be used for relief of the following symptoms: Bone pain Compression of the food pipe (esophagus), windpipe, spinal cord, or superior vena cava caused by tumors Obstructive pneumonia caused by the tumor Treatment of relapse of small-cell lung cancer Persons who have a relapse of small-cell lung cancer have an extremely poor prognosis. If the disease does not respond to treatment or progresses after initial treatment (called "refractory disease") additional treatment may help relieve symptoms and increase survival time somewhat. The most common drug used in this setting is topotecan. Persons whose cancer does not progress for more than three months may be given additional chemotherapy, including re-treatment with their original chemotherapy regimen. Persons with relapsed or refractory small-cell lung cancer may enroll in a clinical trial. For information about ongoing clinical trials, visit the National Cancer Institute's Clinical Trials. Other drugs may be given to prevent and treat adverse effects of radiation, chemotherapy, or the cancer itself, such as nausea or vomiting. Pain medications are also important to relieve any pain due to cancer or its treatment. Surgery Surgery plays little, if any, role in the management of small-cell lung cancer, because almost all cancers have spread by the time they are discovered. The exceptions are the relatively small number of people (less than 15%) whose cancer is discovered at a very early stage of the disease, when the cancer is confined to the lung without any spread to the lymph nodes. However, surgery alone is not considered curative, so chemotherapy is also given. Sometimes radiation therapy will also be needed if the cancer had spread to the nearby lymph nodes. Other Therapy Radiation therapy Radiation therapy is the use of high-dose X-rays or other high-energy rays to kill cancer cells. Radiation can be given from outside the body using a machine (external radiation therapy), or it can be given with the help of radiation-producing materials that are implanted inside the body (brachytherapy). Radiation therapy can be curative (kills all cancer cells), prophylactic (reduces the risk of cancer spreading to the area to which it is given), or palliative (helps reduce suffering). Follow-up Patients who are receiving chemotherapy require close monitoring for side effects and response to therapy. A blood work-up, including CBC (complete blood count), is needed prior to each cycle of chemotherapy to ensure that the bone marrow has recovered before the next dose of chemotherapy is given. Kidney function is monitored, especially if the patient is taking cisplatin, as it can damage the kidneys. Also, carboplatin's dosage is based upon kidney function. The patient will undergo a CT scan to assess their response to treatment Other tests are performed to monitor liver function and electrolytes -- especially sodium and magnesium levels -- due to the effects of the cancer and its treatment. Palliative and terminal care Because small-cell lung cancer is diagnosed in most people when it is not curable, palliative care becomes important. The goal of palliative and terminal care is to manage pain and discomfort and enhance quality of life. Palliative care not only focuses on comfort but also addresses the concerns of the patient's family and loved ones. Caregivers may include family and friends in addition to doctors, nurses, and other health care professionals. Palliative and terminal care is often given in a hospital, hospice, or nursing home; however, it can also be provided at home. The following organizations can help with palliative and terminal care: National Hospice and Palliative Care Organization (800) 658-8898 ( Helpline) Hospice Association of America (202) 546-4759 Hospice Net [email protected] Unlike many other cancers, lung cancer is associated with known risk factors for the disease. The predominant cause of lung cancer is tobacco smoking; therefore, the most important means of preventing lung cancer is to quit smoking. Products that are available to help quit smoking include nicotine gum, medicated nicotine sprays or inhalers, nicotine patches, and oral drugs. In addition, group therapy and behavioral training further increase the chances of quitting. For information about how to quit smoking, visit the following links: American Lung Association, Freedom From Smoking Smokefree.gov Quitnet Other risk factors for lung cancer include asbestos, radon, and uranium exposure. Take precautions to reduce or eliminate exposure to such harmful substances. The success of treatment depends on the stage of small-cell lung cancer. Unfortunately, in most people with small-cell lung cancer, the disease has already spread to other organs of the body by the time it is diagnosed. That shortens life expectancy. The 5-year survival rate is between 2% and 31%. People with small-cell lung cancer in the advanced stage cannot be cured, but treatments are available to improve the quality of life and treat any symptoms of the cancer or its treatment. Support groups and counseling can help you feel less alone and can improve your ability to deal with the uncertainties and challenges that cancer brings. Cancer support groups provide a forum where patients with cancer, survivors of cancer, or both, can discuss the challenges that accompany the illness, as well as guide you in dealing with your concerns. Support groups provide an opportunity to exchange information about the disease, give and take advice about managing side effects, and share feelings with others who are in a similar situation. Support groups also help your family and friends deal with the stress of cancer. Many organizations offer support groups for people with cancer and their loved ones. You can get information about such groups from your doctor, nurse, or hospital social worker. The following organizations can help you with support and counseling: The Lung Cancer Alliance operates a national "phone buddies" program, in addition to other services. (800) 298-2436 [email protected] The National Coalition for Cancer Survivorship is a survivor-led advocacy organization working exclusively on behalf of people with all types of cancer and their families. American Cancer Society American Cancer Society (800) ACS-2345 American Lung Association (800) LUNG- USA (800) 586-4872 National Cancer Institute (800) 4- CANCER (800) 422-6237 American Society of Clinical Oncology (888) 282-2552