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Malignant Pleural Mesothelioma Version 1.2012 Also available at NCCN.com NCCN Guidelines for Patients ® Presented with support from the national law firm of Baron & Budd About this booklet Its purpose Learning that you have cancer can be overwhelming. The goal of this booklet is to help you get the best cancer treatment. It explains which cancer tests and treatments are recommended by experts of malignant pleural mesothelioma. Supported by the NCCN Foundation The NCCN Foundation supports the mission of the National Comprehensive Cancer Network® (NCCN®) to improve the care of patients with cancer. One of its aims is to raise funds to create a library of booklets for patients. Learn more about the NCCN Foundation at www.nccn.com/nccn-foundation. The source of the information NCCN is a not-for-profit alliance of 21 of the world’s leading cancer centers. Experts from NCCN have written treatment guidelines for doctors who treat mesothelioma. These treatment guidelines suggest what the best practice is for cancer care. The information in this booklet is based on these guidelines. For more information This booklet focuses on the treatment of mesothelioma. More information on NCCN, mesothelioma, and other cancers can be found on NCCN.com. © 2012 National Comprehensive Cancer Network, Inc. All rights reserved. The NCCN Guidelines for Patients™ and illustrations herein may not be reproduced in any form for any purpose without the express written permission of NCCN. NCCN Guidelines for Patients®: Malignant Pleural Mesothelioma Version 1.2012 2 Table of contents 4 5 8 10 28 Part 6 – Supportive care Part 2 – Am I at risk? 31 Part 7 – Accepting a treatment plan Part 3 – Testing for mesothelioma 35 Part 8 – Tools How to use this booklet Part 1 – About mesothelioma Explains where malignant pleural mesothelioma starts and how it spreads. Describes what increases your chances for malignant pleural mesothelioma. Explains when you should be tested and the tests used. 15 Part 4 – Treatment planning 18 Part 5 – Treating mesothelioma 44 Describes the tests used to stage mesothelioma and plan treatment. 51 56 Presents the recommended treatment for malignant pleural mesothelioma. 21 S tage I – III epithelioid or mixed mesothelioma 27 S tage I – III sarcomatoid or Stage IV mesothelioma NCCN Guidelines for Patients®: Malignant Pleural Mesothelioma Version 1.2012 3 Presents the recommended treatment for the physical and emotional challenges of mesothelioma. Offers information to help you get a good treatment plan. Presents short reviews of Parts 1 to 7 along with helpful webpages and questions to ask your doctor. Part 9 – Dictionary The definitions of medical and other terms are listed. Credits Index How to use this booklet Who should read this booklet? The recommendations in this booklet include what the NCCN doctors feel is the most useful based on science and their experience. However, these recommendations may not be right for you. Your doctors may suggest other tests or treatments based on your medical history and other factors. This booklet does not replace the knowledge and suggestions of your doctors. Mesothelioma is a rare cancer of the mesothelium—a layer of cells that makes fluid. Mesothelioma can start in the lining of the abdomen (peritoneal mesothelioma), heart (pericardial mesothlioma), testicles (testicular mesothelioma), or lungs (pleural mesothelioma). This booklet focuses on mesothelioma that starts in the lining of the lungs since it is the most common type. This booklet may be helpful for patients, caregivers, and other family and friends dealing with this cancer. Reading this booklet at home may help you absorb what your doctors have said and prepare for treatment. Help! I don’t know these words! In this booklet, many medical words are included that describe cancer, tests, and treatments. These are words that you will likely hear your treatment team use in the months and years ahead. Most of the information may be new to you, and it may be a lot to learn. Don’t be discouraged as you read. Keep reading and review the information. Does the whole booklet apply to me? Parts 1 and 2 review some basics about mesothelioma that may help you understand the cancer better. If you’re unsure if you should be tested for mesothelioma, read Part 3. The information in Parts 4 through 8 is for people who have mesothelioma. This information covers many situations. Thus, not everyone will get every test and treatment listed. Your treatment team can point out what applies to you and give you more information. As you read through this booklet, you may find it helpful to create a list of questions to ask your doctors. NCCN Guidelines for Patients®: Malignant Pleural Mesothelioma Version 1.2012 Words that you may not know are defined in the text or the sidebar. Words with sidebar definitions are underlined when first used on a page. All definitions are listed in the Dictionary in Part 9. Acronyms are also listed in the text or the sidebar. Acronyms are words formed from the first letters of other words. One example is U.S. for United States. 4 about mesothelioma Part 1: About mesothelioma The lungs are covered by a double layer of thin tissue called the pleura. The pleura protects and cushions the lungs. Its inner layer of tissue is called the visceral pleura. This layer is directly attached to the lungs and extends down into the spaces between the lobes of the lungs. The outer layer of the pleura is called the parietal pleura. It lines the chest wall and has contact with many body parts in the chest. See Figure 1. The chest wall is the layer of muscles and bones under the skin in the chest area. The pleura is made of mesothelium and connective tissue. Mesothelium is a single sheet of cells that makes lubricating fluid. Connective tissue supports the mesothelium and supplies it with blood. Connective tissue attaches the pleura to other body parts. There is space between the visceral and parietal pleura called the pleural cavity. It is filled with a small amount of pleural fluid made by the mesothelium. Pleural fluid acts as a lubricant. It helps the two pleura layers slide against each other during breathing. Plural fluid also helps the lungs glide against other organs, such as the heart. The same type of tissue as the pleura is found in other areas of the body. However, it is called other names. The tissue lining around the heart is called the pericardium. The tissue lining around the belly area between the chest and pelvis (abdomen) is called the peritoneum. This booklet is about cancer of the pleural mesothelium. NCCN Guidelines for Patients®: Malignant Pleural Mesothelioma Version 1.2012 Figure 1. Pleural mesothelium Illustration Copyright © 2012 Nucleus Medical Media, All rights reserved. www.nucleusinc.com 6 Part 1 Part 2 Part 1: About mesothelioma Part 3 Malignant pleural mesothelioma is the medical name for cancer that starts in the mesothelium of the lungs. It is also simply called mesothelioma. It isn’t lung cancer. Lung cancer begins inside the lungs. Part 4 Cancer is a disease of cells. Abnormal changes (mutations) in genes can turn normal cells into cancer cells. Genes are instructions in cells for making new cells and controlling how cells behave. Gene mutations cause cancer cells to act differently than normal cells. Unlike normal cells, cancer cells can spread and form tumors in other parts of the body. Cancer that has spread is called a metastasis. Cancer cells often spread through lymph or blood. Lymph is a clear fluid that gives cells water and food. It also has white blood cells that fight germs. Lymph nodes are groups of disease-fighting cells that filter lymph and remove germs. Lymph travels throughout the body in vessels like blood does. As shown in Figure 2, lymph vessels and nodes are found everywhere in the body. Part 8 Illustration Copyright © 2012 Nucleus Medical Media, All rights reserved. www.nucleusinc.com 7 Part 9 NCCN Guidelines for Patients®: Malignant Pleural Mesothelioma Version 1.2012 Figure 2. Lymphatic system Part 7 Part 6 Part 5 Normal cells grow and then divide to form new cells as the body needs them. When normal cells grow old or get damaged, they die. Cancer cells don’t do this. Cancer cells make new cells that aren’t needed and don’t die quickly when old or damaged. Over time, the cancer cells grow and divide enough to form a mass called the primary tumor. If the primary tumor isn’t treated, it can grow and invade nearby tissues and organs, such as the lungs. am I at risk? Asbestos is the only known cause of mesothelioma. Asbestos can break into tiny pieces that may be breathed in or swallowed. The asbestos pieces can then get trapped in the lungs and remain there for years. How asbestos gets into the pleura (the tissue lining around the lungs) and causes normal cells to become cancer cells isn’t fully known. It often takes 20 to 40 years after having had contact with asbestos for mesothelioma to develop. There are no studies that clearly show the length of time exposed to asbestos that is needed to increase the risk for mesothelioma. Some patients with mesothelioma have had no clear exposure to asbestos. This suggests that there may be other causes of the disease. Prior radiation therapy, such as that given for Hodgkin’s lymphoma, may be a cause. Part 7 Part 6 Asbestos is divided into two main groups: serpentine and amphibole asbestos. Serpentine is made of long, curly fibers, whereas amphibole is made of straight, needle-like fibers. There are different types of asbestos within each main group, but the most commonly used include chrysotile, crocidolite, and amosite. 9 Part 9 Part 8 Of these three, chrysotile is the most widely used to make products. Chrysotile is a type of serpentine asbestos. Its long, curly fibers make it less likely to break into pieces and cause mesothelioma. Crocidolite and amosite are types of amphibole asbestos. They are used less frequently because their straight, needle-like fibers are more brittle and likely to break, or fragment. NCCN Guidelines for Patients®: Malignant Pleural Mesothelioma Version 1.2012 Part 2 Part 1 A gene mutation is an abnormal change in the instructions for making and controlling cells. Recent studies show that having a mutation in the BAP1 gene may further increase the risk for mesothelioma in people who have been exposed to asbestos. This gene mutation can be passed down from parents to children. Part 3 However, because they fragment, they are more likely to be breathed in or swallowed and cause mesothelioma. Part 4 Asbestos is a group of minerals made of tiny fibers. It is strong, flexible, and resistant to heat and fire. Because of these traits, asbestos has been used in housing and commercial products, such as roofing and brake pads. Part 5 Part 2: Am I at risk? testing for mesothelioma • Trouble breathing, • Chest CT (computed tomography) with contrast, • Chest pain, • Thoracentesis, • A mass under the skin in the chest, • Pleural biopsy, and • Unplanned weight loss, • Possible SMRP (serum mesothelin-related peptide) blood test • Excessive sweating, • Lasting cough, and Chest CT A CT scan is a type of imaging test. Imaging tests take pictures of the insides of your body. A CT scan takes many pictures of a body part from different angles using x-rays. • Fever Pleural effusion and thickening are two common signs of asbestos exposure. Pleural effusion is the buildup of extra fluid in the pleural cavity (the space between the two pleura layers). It is often the earliest sign of asbestosrelated disease. Pleural thickening is widespread scarring of the pleural tissue. Pleural plaques are concentrated areas of pleural thickening. Pleural plaques are the most common sign of asbestos-related disease, but are not believed to lead to cancer. NCCN Guidelines for Patients®: Malignant Pleural Mesothelioma Version 1.2012 A CT of the chest is recommended for mesothelioma. Your doctors will be able to see if tumors have formed and the size of the tumors. They can also see if the cancer has spread beyond the pleura. A CT scan is easy to undergo. Before the test, you may be asked to stop eating or drinking for several hours. 11 Part 1 Part 2 Part 3 Part 4 Part 5 Recommended tests Part 6 Symptoms of mesothelioma Part 7 To confirm if you have mesothelioma you will need to be tested. The recommended tests are listed and described next. These tests are used to find mesothelioma, see how far the primary tumor has spread, and plan treatment. Part 8 It is recommended that you get tested for mesothelioma if you have had pleural effusion more than once, have pleural thickening, or both. Testing for mesothelioma is done when certain symptoms that may be caused by the cancer appear. Symptoms of mesothelioma may not show until decades after contact with asbestos. Symptoms of mesothelioma overlap with symptoms of other health conditions, so only your doctor will be able to tell if your symptoms are due to the cancer. Possible symptoms of mesothelioma include, but aren’t limited to: Part 9 Part 3: Testing for mesothelioma Part 3: Testing for mesothelioma You also should remove any metal objects that are on your body. You will likely be asked to change into a hospital gown to wear during the test. pleural cavity. See Figure 4. For thoracentesis, your skin will be cleaned and numbed with local anesthesia. Next, a needle will be inserted between your ribs into your chest to get the fluid. You may be given an x-ray before the biopsy to guide the needle into your chest and afterward to check if the biopsy went well. The fluid will then be sent to a lab to be tested for cancer cells and biomarkers of cancer, such as cytokeratin. A contrast dye may be used to make the pictures clearer. The dye will be injected into your vein. It may cause you to feel flushed or get hives. Rarely, serious allergic reactions occur. Tell your doctor if you have had bad reactions before. As shown in Figure 3, a CT machine is large and has a tunnel in the middle. During the test, you will need to lie on a table that moves slowly through the tunnel. Pillows or straps may be used to keep you still during the test. You will be alone, but a technician will operate the machine in a nearby room. He or she will be able to see, hear, and speak with you at all times. As the machine takes pictures, you may hear buzzing, clicking, or whirring sounds. Earplugs are sometimes worn to block these sounds. A computer combines all pictures into one detailed picture. A CT scan is done in about 30 seconds, but the entire process is completed in 30 minutes. You may not learn of the results for a few days since a radiologist needs to see the pictures. Thoracentesis To confirm if there’s cancer, a sample of tissue or fluid must be removed from your body. The removal of a tissue or fluid sample is called a biopsy. Thoracentesis is a type of biopsy that removes a fluid sample from the Figure 3. CT scan NCCN Guidelines for Patients®: Malignant Pleural Mesothelioma Version 1.2012 12 Local anesthesia: A controlled loss of feeling in a small area of the body from drugs Pleura: The tissue lining around the lungs Pleural cavity: The space between the two pleura layers Figure 4. Thoracentesis Illustration Copyright © 2012 Nucleus Medical Media, All rights reserved. www.nucleusinc.com NCCN Guidelines for Patients®: Malignant Pleural Mesothelioma Version 1.2012 13 Radiologist: A doctor who’s an expert in reading imaging tests Part 4 Part 3 Part 2 Part 1 Cytokeratin: Type of protein found on cells that line the inside and outside surfaces of the body Part 5 Biomarker: A substance found in body fluid or tissues that may be a sign of cancer Part 6 Allergic reaction: Symptoms caused when the body is trying to rid itself of invaders Part 7 A CT-guided core biopsy uses pictures from a CT scan to guide a needle into your body. First, your skin will be cleaned and numbed with local anesthesia. Next, the needle will be inserted through a small cut and guided into your chest between your ribs. Then, the sample will be removed by the sharp end of the needle and stored in a hollow space within the needle. Definitions Part 8 Pleural biopsy A pleural biopsy removes one or more samples of the pleura. The samples are then sent to a lab to be tested for cancer cells and biomarkers of cancer, such as cytokeratin. There are a few ways that a pleural biopsy can be done. Options are a CT-guided core biopsy, an Abrams needle biopsy, an open biopsy, or a thoracoscopic biopsy. Thoracoscopic biopsy is the preferred method. Part 9 Part 3: Testing for mesothelioma Part 3: Testing for mesothelioma An Abrams needle biopsy is like a core biopsy. However, it may or may not use CT. An Abrams needle limits the amount of air that enters tissue during a pleural biopsy. An open biopsy requires a large surgical cut into the chest. General anesthesia, a controlled loss of wakefulness from drugs, is used. This cut allows the surgeon to see into your chest without any tools. Samples are collected with a surgical knife. For this biopsy, you will have to stay in the hospital overnight. For a thoracoscopic biopsy, also called a VATS (videoassisted thoracoscopic surgery) biopsy, a small cut into your chest is needed. General anesthesia is used. Next, a thoracoscope is inserted through the cut. A thoracoscope is a thin, tube-shaped instrument that has a light and camera for viewing as well as a tool to remove a sample. This surgery may cause some pain and swelling and will leave a small scar. SMRP blood test Mesothelin-related peptides are made when protein in the mesothelium breaks down. Mesothelium is a single layer of cells that makes lubricating fluid. These breakdown products are found in blood (serum). People with mesothelioma often have high levels of SMRP. This test requires a sample of blood to be drawn from a vein in your arm. The sample is then sent to the lab to be tested. NCCN Guidelines for Patients®: Malignant Pleural Mesothelioma Version 1.2012 14 treatment planning Part 4: Treatment planning Chest and abdominal CT If you have not already had a chest CT scan, it should be done along with a CT of your abdomen. Contrast should be used if possible. Contrast is a dye put into your body to make clearer pictures during imaging tests. These scans can show your doctors where the tumor is, how big it is, and if it has spread. See page 11 for more information about CT scans. Based on the tests in Part 3, your doctors will let you know if you have mesothelioma. If you do, a team of experts with experience treating mesothelioma should plan your treatment. Your team may include a: • Pulmonologist - expert in treating lung diseases, • Diagnostic imaging specialist - expert in imaging tests, • Radiation oncologist - expert in radiation treatment, • Medical oncologist - expert in cancer drugs, and PET/CT Like CT, PET is an imaging test that takes pictures of the inside of your body. However, instead of showing the shape of an organ or tumor, it shows how your cells are using a simple form of sugar. To create the pictures, a radiotracer first needs to be put into your body. A radiotracer is matter with energy that is put into your body to make pictures clearer. For mesothelioma, the radiotracer used is FDG (18F-fluorodeoxyglucose). FDG is made of fluoride and glucose (sugar). Cancer cells use more FDG than normal cells and thus show up as bright spots on pictures. For this test, you must fast for 4 hours or more. PET is often used with CT. FDG PET/CT is helpful for finding mesothelioma that has spread to lymph nodes or distant sites. This test should be done before talc pleurodesis. See page 29 for more information about talc pleurodesis. • Surgeon - expert in operations to remove or repair a part of the body. Once mesothelioma is confirmed, your doctors will need to know the stage of the cancer. The cancer stage is a rating by your doctors, based on tests, of how far the cancer has grown and spread. It is used to plan which treatments are best for you. The recommended tests for cancer staging are: Recommended tests • Chest and abdominal CT with contrast, • PET/CT (positron emission tomography/ computed tomography), • Mediastinoscopy or EBUS-FNA (endobronchial ultrasound–guided fine-needle aspiration), Mediastinoscopy If your treatment may include surgery, a biopsy of the lymph nodes in the mediastinum is often recommended. The mediastinum is the area of the chest between the lungs. • Possible chest MRI (magnetic resonance imaging), and • Possible VATS and/or laparoscopy NCCN Guidelines for Patients®: Malignant Pleural Mesothelioma Version 1.2012 16 Diaphragm: A sheet of muscles below the ribs that helps a person to breathe VATS and laparoscopy VATS is a type of biopsy, also called thoracoscopic biopsy. At least two small cuts are made between your ribs into your chest. A thoracoscope to see inside your chest is inserted through one cut. Surgical tools are inserted through the other cuts to remove samples of the pleura, lymph nodes, or other tissue. Laparoscopy is a type of surgery to view and take biopsy samples of organs and tissue in the abdomen (belly). A laparoscope is inserted through a tiny cut in your abdomen. A laparoscope is a thin, lighted tube with a lens for viewing and tools to remove samples of tissue. These procedures are recommended if imaging tests suggest that there is mesothelioma in the pleura (tissue lining the lungs) on both sides of your chest or in the peritoneum (tissue lining the abdomen). NCCN Guidelines for Patients®: Malignant Pleural Mesothelioma Version 1.2012 17 Lymph nodes: Small groups of disease-fighting cells located throughout the body Thoracoscope: Thin tube with a light, lens, and tools to see and remove samples of tissue from the chest Ultrasound: A test that uses sound waves to take pictures of inside the body Part 3 Part 2 Part 1 MRI MRI is an imaging test that uses radio waves and powerful magnets. It shows soft tissues better than a CT scan. An MRI scan of your chest can show if the cancer has spread to your chest wall, spine, diaphragm, or blood vessels. The scan may cause your body to feel a bit warm. Like a CT scan, a contrast dye may be used. Chest wall: The layer of muscles and bones under the skin covering the chest Part 4 Bronchoscope: Thin, long tube fitted with tools that is guided down the throat Part 5 EBUS-FNA This is another way to perform a biopsy of the mediastinal lymph nodes. However, EBUS-FNA doesn’t require any surgical cuts. During an EBUS-FNA, a bronchoscope attached to an ultrasound device is guided down your throat into your lungs. The ultrasound is used to find the lymph nodes. Next, a very thin needle is passed through the wall of a main airway of the lungs and into a lymph node to get a sample. Part 6 Biopsy: Removal of small amounts of tissue or fluid to be tested for disease Part 7 Definitions Part 8 Samples of these lymph nodes can be obtained by mediastinoscopy. For this biopsy, a mediastinoscope is inserted through a small cut in your neck right above your sternum. A mediastinoscope is a thin, tube-shaped instrument that has a light and camera for viewing as well as a tool to remove a sample. General anesthesia is used. General anesthesia is a controlled loss of wakefulness from drugs. This surgery may cause some pain and swelling and will leave a small scar. Part 9 Part 4: Treatment planning treating mesothelioma The tumor has grown into either the diaphragm or the lung. Stage III • The tumor has grown into 1) the first layer of the chest wall or a confined area of the other layers, 2) the fat between the lungs, or 3) the pericardium, and/or • The cancer has spread to nearby lymph nodes or the nodes at the base of the breast bone. Stage IV • The tumor has grown into 1) a wide area of the chest wall, 2) the peritoneum, 3) an organ between the lungs, 4) the spine, or 5) the pleura around the other lung, • The cancer has spread to the lymph nodes near the other lung or near the collarbone, and/or • The cancer has spread to distant sites. NCCN Guidelines for Patients®: Malignant Pleural Mesothelioma Version 1.2012 19 Pericardium: The tissue lining around the heart Peritoneum: The tissue lining around the organs in the belly area Pleura: The tissue lining around the lungs Spine: Bones, muscles, and other tissues along the back from the base of the skull to the tailbone Part 3 Part 2 Part 1 Lymph nodes: Small groups of special diseasefighting cells located throughout the body Part 4 Stage II Part 5 The tumor hasn’t grown beyond the pleura where it started. Diaphragm: A sheet of muscles below the ribs that helps a person to breathe Part 6 Stage I Chest wall: The layer of muscles and bones under the skin covering the chest Part 7 Stages of mesothelioma Definitions Part 8 The recommended treatment for mesothelioma is based on two features of the cancer. One of these features is the clinical stage. The clinical stage is a rating of the extent of a tumor based on tests before treatment. The AJCC (American Joint Committee on Cancer) staging system is most often used for mesothelioma. In this system, there are four cancer stages based on the growth and spread of the cancer described below: Part 9 Part 5: Treating mesothelioma Part 5: Treating mesothelioma The second feature used to recommend treatment is the type of mesothelioma cell. A pathologist will classify the cell type based on the size, shape, and structure of the cells. This is known as histologic subtyping. The three types of mesothelioma cells are: Mesothelioma cell types Epithelioid The cells are uniform and form complex, organized patterns. Sarcomatoid The cells are spindle-shaped and form disorganized patterns. Biphasic (mixed) The cells are both epithelioid and sarcomatoid cells. Stages I – III with either an epithelioid or mixed cell type are treated the same. The recommended treatments for these mesotheliomas can be found starting on page 21. Stage IV mesothelioma and any mesothelioma with a sarcomatoid cell type are treated the same. See page 27 for treatment recommendations. Definitions Definitions: Artery: A tube that carries blood from the heart to organs throughout the body Observation: A period of testing to watch for when to start treatment based on cancer growth Oxygen: A gas in the air that the body needs to live Pathologist: A doctor who’s an expert in testing cells to find disease Radiotracer: Matter with energy that is put into the body to make pictures clearer Side effect: An unplanned physical or emotional response to treatment Surgery: An operation to remove or repair a part of the body Tumor: An overgrowth of cells NCCN Guidelines for Patients®: Malignant Pleural Mesothelioma Version 1.2012 20 Cardiac stress tests Cardiac stress tests assess how well your heart works while exercising. Some heart problems are easier to find when your heart is working hard. Before the test, several small patches will be placed on your chest. The patches are connected to an ECG (electrocardiography) machine by long wires. The ECG machine measures the electrical activity of your heart during the test. A blood pressure cuff may be fitted on your arm too. Next, your heart activity will be recorded while you rest then as you exercise. The level of exercise will be increased until you are unable to keep going. After the test, you will be watched until your heart activity and blood pressure return to normal. • Possible perfusion scanning, and • Cardiac stress test Pulmonary function tests These are tests that show how well your lungs work. You may be asked not to eat much, not to smoke, and not to take some medications before testing. A common side effect of these tests is shortness of breath. Spirometry involves blowing into a tube to measure how much air and how fast you breathe. A gas diffusion test involves breathing in a harmless gas and measuring how much you breathe out. It tells how much oxygen travels from your lungs into your blood. A body plethysmograph involves sitting in a small room and breathing into a tube. This test measures how much air your lungs can hold and how much air is in your lungs after you exhale. Next steps: If surgery is still an option, read the next page for treatment recommendations. If surgery isn’t possible, you have two options. One option is to start observation if you have no symptoms of the cancer. The second option is to start treatment without delay. See page 27 to read about treatment options other than surgery. Perfusion scanning Perfusion scanning shows the blood flow in and out of your lungs. You may have this test if the pulmonary NCCN Guidelines for Patients®: Malignant Pleural Mesothelioma Version 1.2012 21 Part 1 Part 2 Part 3 Part 4 • Pulmonary function tests, Part 5 Recommended tests Part 6 function tests suggest your lungs aren’t working well. First, a radiotracer will be injected into your arm. Next, a camera that detects the radiotracer will take pictures of your chest. In normal lungs, the radiotracer will be evenly spread throughout the lungs. Areas without the radiotracer may be caused by a blocked artery, tumor, or other health problems. Part 7 Surgery may be a treatment option. Your doctors will use the tests described in Parts 3 and 4 to assess if the tumor can be surgically removed. Also, your doctors must assess the health of your heart and lungs. Part 8 Stage I – III epithelioid or mixed mesothelioma Part 9 Part 5: Treating mesothelioma Part 5: Treating mesothelioma Stage I – III epithelioid or mixed mesothelioma Treatment with surgery Definitions: Neoadjuvant treatment Tests Primary treatment Adjuvant treatment Pemetrexed + cisplatin • Chest CT, and • Possible other imaging tests P/D (pleurectomy/decortication) None EPP (extrapleural pneumonectomy) Radiation therapy P/D (pleurectomy/decortication) Chemotherapy EPP (extrapleural pneumonectomy) Chemotherapy + radiation therapy None Neoadjuvant treatment You can receive chemotherapy either before or after surgery. Chemotherapy given before surgery is called neoadjuvant treatment. It is given to shrink the tumor before it is removed from the body. Pemetrexed with cisplatin is recommended for neoadjuvant treatment. Chemotherapy is given in cycles of treatment days followed by days of rest. Cycles give the body a chance to recover before the next treatment. The length of 1 cycle of pemetrexed and cisplatin is 21 days. Pemetrexed and cisplatin are given on Day 1 of the cycle. Both drugs are liquids that are slowly injected into your vein. Chemotherapy is the use of drugs to treat cancer. Chemotherapy stops the growth process of cells in an active growth phase. It doesn’t work on cells in a resting phase. Cancer cells grow fast, so chemotherapy works well to stop new cancer cells from being made. However, it also kills normal cells and can cause side effects. Side effects are unplanned physical or emotional responses to treatment. Ask your doctor for a list of side effects caused by chemotherapy. Tests If you receive neoadjuvant treatment, a CT scan of your chest is recommended to check treatment results. The CT scan can show if the tumor is smaller and where it Acronyms is still present. See page 11 for more information on CT scans. Based on the chest CT, your doctors may want you to have another imaging test to assess if there’s cancer in the area between your lungs. NCCN Guidelines for Patients®: Malignant Pleural Mesothelioma Version 1.2012 22 There are two types of surgery for mesothelioma: P/D (pleurectomy/ decortication) or EPP (extrapleural pneumonectomy). P/D removes the tumor and the pleura affected by the tumor. EPP removes both pleural layers, the lung and diaphragm on the same side of the chest as the tumor, and often the pericardium. During both surgeries, lymph node sampling should be done to assess if the cancer has spread to the lymph nodes between your lungs. Lymph node sampling only removes some of the nodes from a cluster. Lymph nodes: Small groups of disease-fighting cells located throughout the body There is no well-designed research to show which surgery is better in which circumstances. Your doctors will recommend a surgery based on many factors. They will consider your overall health, ability to do activities, symptoms, cancer stage, and the type of mesothelioma cell. They will also consider that the dangers of surgery are more likely and more serious with EPP than P/D. Ask your treatment team for a list of all the dangers of the surgery they recommend. Radiation therapy: The use of radiation to treat cancer NCCN Guidelines for Patients®: Malignant Pleural Mesothelioma Version 1.2012 23 Diaphragm: A sheet of muscles below the ribs that helps a person to breathe Pericardium: The tissue lining around the heart Acronyms CT = Computed tomography Part 3 Part 2 Part 1 Cancer stage: A rating of the growth and spread of the cancer Part 4 Before surgery, your surgeon will perform a surgical exploration. This is a minor surgery to look inside your chest to see how much the tumor has grown and spread. Use of a thoracoscope inserted through one surgical cut is the preferred method for exploration. A thoracoscope is a thin, tube-shaped instrument with a light, camera lens, and tools on the end for viewing and to remove tissue from the chest. If the whole tumor can’t be removed, surgery isn’t recommended. Part 5 Board-certified: A status to identify doctors who are trained in a specialized field of medicine Part 6 Definitions Part 7 Primary treatment Primary treatment is the main treatment used to rid your body of cancer. Surgery is used as a primary treatment for some mesotheliomas. Surgery should be performed by a board-certified thoracic surgeon. A thoracic surgeon is a doctor who’s an expert in operating on organs inside the chest. Part 8 Stage I – III epithelioid or mixed mesothelioma Part 9 Part 5: Treating mesothelioma Part 5: Treating mesothelioma Stage I – III epithelioid or mixed mesothelioma Adjuvant treatment Adjuvant treatment is given after surgery to treat any remaining cancer cells. Adjuvant treatment after P/D is chemotherapy. If you had chemotherapy before P/D, no adjuvant treatment is recommended. See page 27 for a list of chemotherapy drugs for mesothelioma. After EPP, radiation therapy is recommended. Chemotherapy may also be given if not given before surgery. Radiation therapy uses high-energy rays to treat cancer. The rays damage a cell’s instructions for creating and controlling cells. This either kills the cancer cells or stops new cancer cells from being made. Radiation can also harm normal cells. Ask your treatment team for a list of all the side effects of radiation. Radiation will be given to the side of your chest where the tumor was and other areas decided by your radiation oncologist. For mesothelioma, radiation is often given using a machine outside the body. This method is called EBRT (external beam radiation therapy). Notes _____________________ _____________________ _____________________ _____________________ _____________________ _____________________ _____________________ _____________________ _____________________ _____________________ Before radiation, images of the chest are taken with a CT scan using contrast. This process is called simulation. Your radiation oncologist will use the images to decide the radiation dose and to shape the radiation beams. Beams are shaped with computer software and hardware added to the radiation machine. _____________________ Your treatment team will decide the best time to have radiation therapy after surgery. During treatment, you will lie on a table in the same position as done during simulation. Devices may be used to keep you from moving so that the radiation targets the tumor. Likewise, methods may be applied to control breathing. _____________________ _____________________ _____________________ _____________________ _____________________ _____________________ _____________________ NCCN Guidelines for Patients®: Malignant Pleural Mesothelioma Version 1.2012 24 Side effect: An unplanned physical or emotional response to treatment Acronyms CT = Computed tomography EPP = Extrapleural pneumonectomy P/D = Pleurectomy/ decortication NCCN Guidelines for Patients®: Malignant Pleural Mesothelioma Version 1.2012 25 Part 4 Part 3 Part 2 Part 1 Radiation oncologist: A doctor who’s an expert in treating cancer with radiation Part 5 Contrast: A dye put into your body to make clearer pictures during imaging tests Part 6 One session can take between 30 to 60 minutes. You will likely have 5 sessions a week. Radiation therapy lasts 4 to 5 weeks if no cancer cells were found in the normal-looking tissue around the tumor removed during surgery. If cancer is found, radiation therapy lasts about 5 to 6 weeks. Chemotherapy: Drugs that stop the growth process of cells in an active growth phase Part 7 You will be alone while the technician operates the machine from a nearby room. He or she will be able to see, hear, and speak with you at all times. As treatment is given, you may hear noises. Definitions Definitions: Part 8 Stage I – III epithelioid or mixed mesothelioma Part 9 Part 5: Treating mesothelioma Part 5: Treating mesothelioma Stage I – III epithelioid or mixed mesothelioma The main types of EBRT used for mesothelioma include: • 3D-CRT (three-dimensional conformal radiation therapy) – Radiation is given in small doses for a few weeks with x-ray beams that match the shape of the tumor, • IMRT (intensity-modulated radiation therapy) – Radiation is given in small doses for a few weeks with x-ray beams of different strengths based on the thickness of the tumor, • Tomotherapy – A type of IMRT, a tomotherapy machine takes CT scans and delivers radiation, and Definitions Conformal radiation therapy: Radiation beams are shaped to match the shape of the cancer using imaging scans EBRT: Radiation therapy received from a machine outside the body • Proton beam therapy – Radiation is given using proton beams, which deliver more radiation at the end rather than throughout the beam. First-line treatment: The first set of treatments given to treat a disease Conformal radiation therapy is the preferred method by NCCN doctors. IMRT, tomotherapy, and proton beam therapy are newer radiation therapy methods. They should only be used in experienced cancer centers or according to a specific, detailed treatment plan called a protocol. Second-line treatment: The next treatment(s) given when first-line treatment fails Side effect: An unplanned physical or emotional response to treatment Acronyms CT = Computed tomography EBRT = External beam radiation therapy NCCN Guidelines for Patients®: Malignant Pleural Mesothelioma Version 1.2012 26 Chemotherapy First-line treatment Second-line treatment Pemetrexed + cisplatin (preferred) Pemetrexed Pemetrexed + carboplatin Vinorelbine Gemcitabine + cisplatin The length of 1 cycle of gemcitabine and cisplatin is 21 to 28 days. For each cycle, gemcitabine is given on Days 1, 8, and 15, and cisplatin is given only on Day 1. Vinorelbine is given 1 time every 7 days. If the cancer grows or spreads after first-line treatment, then you may receive second-line treatment. However, there is little research to provide guidelines for secondline treatment. If given as first-line treatment, pemetrexed should only be given as second-line treatment if a good, lasting response was achieved with it the first time. Gemcitabine Pemetrexed Vinorelbine NCCN Guidelines for Patients®: Malignant Pleural Mesothelioma Version 1.2012 27 Part 1 Part 2 Part 3 Part 4 Part 5 Chemotherapy is given in cycles of treatment days followed by days of rest. Cycles give the body a chance to recover before the next treatment. The length of 1 cycle of pemetrexed and cisplatin is 21 days. Pemetrexed and cisplatin are given on Day 1 of the cycle. Other treatments that also follow this cycle include pemetrexed with carboplatin and pemetrexed alone. Part 6 Cisplatin with pemetrexed is preferred for first-line treatment. But, gemcitabine with cisplatin is a good option if you cannot take pemetrexed. And pemetrexed with carboplatin is a good option if you have poor overall health, difficulty doing daily activities, or other serious health conditions. Part 7 Chemotherapy drugs stop the growth process of cells in an active growth phase. Cancer cells grow fast, so chemotherapy works well to stop new cancer cells from being made. However, it also kills normal cells and can cause side effects. Ask your doctor for a list of all the side effects caused by the chemotherapy drugs you will have. All of the chemotherapy drugs listed in the table below are liquids that are slowly injected into your vein. Part 8 Stage I – III sarcomatoid or Stage IV mesothelioma Part 9 Part 5: Treating mesothelioma supportive care • Distress Pleural effusion Pleural effusion is the buildup of extra fluid in the pleural cavity. This can result in shortness of breath and make it hard to breathe. A pleural catheter can be used to drain the fluid buildup. Another treatment is talc pleurodesis. Talc pleurodesis involves putting talc powder into the pleural cavity. The powder irritates the tissue and causes the pleura layers to grow together while healing. This seals the pleural cavity and stops fluid buildup. It is important that a PET/CT scan is done before talc pleurodesis because talc causes pleural inflammation, which can look like cancer on the scan. Chest pain Chest pain may be caused by cancer growing into the chest wall. Radiation therapy may be used to ease chest pain. However, there are other ways to manage chest pain such as with pain medications or chemotherapy. For more information about chest pain and management, talk to your treatment team. 29 Pleura: The tissue lining around the lungs Pleural cavity: The space between the two pleura layers Acronyms PET/CT = Positron emission tomography/ computed tomography Part 4 Part 3 Part 2 Part 1 Inflammation: Redness, heat, pain, and swelling from injury or infection • Smoking cessation, and Part 5 • Chest pain, Part 6 Chest wall: The layer of muscles and bones under the skin that covers the chest area • Pleural effusion, NCCN Guidelines for Patients®: Malignant Pleural Mesothelioma Version 1.2012 Catheter: A thin, flexible tube used to give or drain fluids from the body Part 7 Conditions for supportive care Definitions Definitions: Part 8 For most patients with cancer, their main concern is that their treatment works. However, having cancer is complex and brings many physical and emotional challenges. It is important to know about these challenges and get the support you need. Supportive care is the treatment of symptoms of a disease. Part 6 lists some of the challenges you may face as well as ways to manage them. Part 9 Part 6: Supportive care Part 6: Supportive care Smoking cessation Smoking can worsen your overall health and how well your lungs work. If you are a smoker, it is important to talk with your treatment team about ways to quit. It may also be very helpful to join a support group. depression during a hard part of treatment. Feeling distressed may be a minor problem, or it may be more serious. Serious or not, tell your treatment team so that you can get help if needed. Help can include support groups, “talk” therapy, or medication. At your cancer center, cancer navigators, social workers, and other experts can help. Some people also feel better by exercising, talking with loved ones, or relaxing. Distress Many patients with cancer experience symptoms of distress, such as anxiety and depression. You may feel anxious during testing, or you may have a passing NCCN Guidelines for Patients®: Malignant Pleural Mesothelioma Version 1.2012 30 accepting a treatment plan Part 7: Accepting a treatment plan Parts of a treatment plan Having cancer is very stressful. While absorbing the fact that you have cancer, you must learn about tests and treatments. In addition, the time you have to accept a treatment plan feels short. Parts 1 through 6 aimed to teach you about pleural mesothelioma. Part 7 addresses issues related to accepting a treatment plan. A treatment plan addresses all cancer care needs while respecting your beliefs, wishes, and values. It is likely to change and expand as you go through treatment. The plan will include the role of your doctors and how you can help yourself. A treatment plan often has the following parts: Benefits of a treatment plan A treatment plan is a written course of action through treatment and beyond. It can help you, your loved ones, and your treatment team. A treatment plan is useful for: Cancer information Cancer can greatly differ even when people have a tumor in the same organ. Test results that describe the cancer are reported in the treatment plan. Such test results include the cancer site, mesothelioma cell type, and cancer stage. • Starting and guiding talks about treatment, • Teaching what the treatment choices are, • Informing everyone of the decisions made, Your treatment team Pleural mesothelioma should be treated by a multidisciplinary team with experience in mesothelioma. Your treatment team may include a radiation oncologist, thoracic surgeon, medical oncologist, diagnostic imaging specialist, and pulmonologist. Your primary care doctor can also be part of your team. Besides doctors, you may receive care from nurses, social workers, and other health experts. Ask to have the names and contact information of your health care providers included in the treatment plan. • Pinpointing who is in charge of each part of care, • Controlling stress, • Knowing what to expect, • Changing from one doctor to another, • Improving contact among your doctors, and • Providing care for all issues. NCCN Guidelines for Patients®: Malignant Pleural Mesothelioma Version 1.2012 32 • Treatment side effects, • Costs of treatment, and • What you want from treatment. The cancer treatment that you agree to have should be reported in the treatment plan. It is also important to note the goal of treatment and the chance of a good treatment result. In addition, all known side effects should be listed and the time required to treat them should be noted. Your treatment plan may change because of new information. You may change your mind about treatment. Tests may find new results. How well the treatment is working may change. Any of these changes may require a new treatment plan. Stress and symptom control Cancer and its treatment can cause bothersome symptoms. You may also have symptoms from the stress of having cancer. Such symptoms include pain, sleep loss, and anxiety. There are ways to treat many symptoms, so tell your treatment team about any symptoms you have so they can help. Financial stress is common. You may be unemployed or miss work during treatment. You may have too little or no health insurance. Discuss these issues with your treatment team and they will include information in the treatment plan to help you control your finances. NCCN Guidelines for Patients®: Malignant Pleural Mesothelioma Version 1.2012 33 Pulmonologist: A doctor who’s an expert in treating lung diseases Radiation oncologist: A doctor who’s an expert in treating cancer with radiation Side effect: An unplanned physical or emotional response to treatment Thoracic surgeon: A doctor who’s an expert in operating on organs inside the chest Part 3 Part 2 Part 1 Medical oncologist: A doctor who’s an expert in cancer drugs Part 4 • Your general health, Part 5 Diagnostic imaging specialist: A person trained to read pictures of the body from imaging tests Part 6 • Location of the cancer, Cancer stage: Rating of the growth and spread of the cancer Part 7 • The cancer stage and cell type, Definitions Definitions: Part 8 Cancer treatment There is no single treatment practice that is best for all patients. There is often more than one treatment option. Treatment planning takes into account many factors, such as: Part 9 Part 7: Accepting a treatment plan Part 7: Accepting a treatment plan Advanced care Talking with your doctor about your prognosis can help with treatment planning. If the cancer can’t be cured, a care plan for the end of life can be made. However, such talks often happen too late or not at all. Your doctor may delay these talks for fear that you may lose hope, become depressed, or have a shorter survival. Studies suggest that these fears are wrong. Instead, there are many benefits to advanced care planning. It is useful for: Hospice care allows you to have the best quality of life possible. Care is given all day, every day of the week. You can choose to have hospice care at home or at a hospice center. One study found that patients and caregivers had a better quality of life when hospice care was started early. An advance directive describes the treatment you’d want if you weren’t able to make your wishes known. It also can name a person whom you’d want to make decisions for you. It is a legal paper that your doctors have to follow. It can reveal your wishes about life-sustaining machines, such as feeding tubes. It can also include your treatment wishes if your heart or lungs were to stop working. If you already have an advance directive, it may need to be updated to be legally valid. • Knowing what to expect, • Making the most of your time, • Lowering the stress of caregivers, • Having your wishes followed, • Having a better quality of life, and Getting a 2nd opinion • Getting good care. The time around a cancer diagnosis is very stressful. People with cancer often want to get treated as soon as possible. While cancer can’t be ignored, there is time to think about and choose which treatment plan is best for you. You may wish to have another doctor review your test results and the treatment plan your doctor has recommended. This is called getting a 2nd opinion. Advanced care planning starts with an honest talk between you and your doctors. You don’t have to know the exact details of your prognosis. Just having a general idea will help with planning. With this information, you can decide at what point you’d want to stop cancer treatment, if at all. You can also decide what treatments you’d want for symptom relief. Copies of all test results need to be sent to the doctor giving the 2nd opinion. Some people feel uneasy asking for copies from their doctors. However, a 2nd opinion is a normal part of cancer care. What’s more, some health plans require a 2nd opinion. If your health plan doesn’t cover the cost of a 2nd opinion, you have the choice of paying for it yourself. Another part of the planning involves hospice care. Hospice care doesn’t include treatment to fight the cancer but rather to reduce symptoms caused by cancer. Hospice care may be started because you aren’t interested in more cancer treatment, no other cancer treatment is available, or because you may be too sick for cancer treatment. NCCN Guidelines for Patients®: Malignant Pleural Mesothelioma Version 1.2012 34 Part 1 Review of Part 1 – About mesothelioma • The pleural mesothelium is part of the tissue lining around the lungs. • Malignant pleural mesothelioma is cancer that starts in the pleural mesothelium. Part 3 Part 2 Part 8: Tools American Cancer Society www.cancer.org/Cancer/MalignantMesothelioma/DetailedGuide/malignant-mesothelioma-malignant-mesothelioma National Cancer Institute www.cancer.gov/cancertopics/pdq/treatment/malignantmesothelioma/patient/page1#Keypoint1 NCCN Guidelines for Patients®: Malignant Pleural Mesothelioma Version 1.2012 35 Part 9 Part 8 Part 7 Mesothelioma Applied Research Foundation www.curemeso.org/site/c.kkLUJ7MPKtH/b.8050629/k.6568/What_is_Mesothelioma.htm Part 5 Webpages Part 6 • Mesothelioma cells can spread to other body parts through lymph or blood. Part 4 • Mesothelioma cells form a tumor since they don’t die as they should. Part 8: Tools Review of Part 2 – Risk factors • Asbestos is a group of minerals made of tiny fibers that are strong and flexible. • Asbestos can break into tiny pieces that may be breathed in or swallowed. • Asbestos is the only known cause of mesothelioma. Webpages American Cancer Society www.cancer.org/Cancer/MalignantMesothelioma/OverviewGuide/malignant-mesothelioma-overview-risk-factors National Cancer Institute www.cancer.gov/cancertopics/factsheet/Risk/asbestos Mesothelioma Applied Research Foundation www.curemeso.org/site/c.kkLUJ7MPKtH/b.8050905/k.7461/What_Causes_Mesothelioma.htm CURE magazine http://media.curetoday.com/downloads/documents/pocketguide_mesothelioma.pdf NCCN Guidelines for Patients®: Malignant Pleural Mesothelioma Version 1.2012 36 Part 1 • Pleural effusion is the buildup of extra fluid between the two layers of tissue lining the lungs. • Pleural thickening is widespread scarring of the tissue around the lungs. Part 2 Review of Part 3 – Testing for mesothelioma Part 3 Part 8: Tools American Cancer Society www.cancer.org/Cancer/MalignantMesothelioma/DetailedGuide/malignant-mesothelioma-diagnosed National Cancer Institute www.cancer.gov/cancertopics/pdq/treatment/malignantmesothelioma/patient#Keypoint3 NCCN Guidelines for Patients®: Malignant Pleural Mesothelioma Version 1.2012 37 Part 9 Part 8 Part 7 Mesothelioma Applied Research Foundation www.curemeso.org/site/c.kkLUJ7MPKtH/b.8055879/k.705B/Mesothelioma_Diagnosis.htm Part 5 Webpages Part 6 • Imaging scans and tests of pleural fluid and tissue are needed to confirm if you have mesothelioma. Part 4 • Get tested for mesothelioma if you have had pleural effusion, pleural thickening, or both. Part 8: Tools Questions about testing to ask your doctor • What tests will I have? • Should I bring a list of my medications? • Where will the tests take place? Will I have to go to the hospital? • Should I bring someone with me? • How long will it take for me to recover? Will I be given an antibiotic or other drug afterward? • How long will it take? Will I be awake? • Will it hurt? Will I need anesthesia? • How soon will I know the results and who will explain them to me? If a biopsy is done, will I get a copy of the results? • What are the risks? What are the chances of infection or bleeding afterward? • Who will talk with me about the next steps? When? • How do I prepare for testing? Should I not take aspirin? Should I not eat beforehand? NCCN Guidelines for Patients®: Malignant Pleural Mesothelioma Version 1.2012 38 Part 1 Review of Part 4 – Treatment planning • Cancer staging is a rating of how far the cancer has grown and spread. • The cancer stage is used to plan treatment. Part 3 Part 2 Part 8: Tools Part 4 • Cancer tests are used to determine the cancer stage. National Cancer Institute www.cancer.gov/cancertopics/pdq/treatment/malignantmesothelioma/patient/page2#Keypoint6 NCCN Guidelines for Patients®: Malignant Pleural Mesothelioma Version 1.2012 39 Part 9 Part 8 Part 7 Mesothelioma Applied Research Foundation www.curemeso.org/site/c.kkLUJ7MPKtH/b.8055915/k.3AAE/Mesothelioma_Staging_and_Outcomes.htm Part 6 American Cancer Society www.cancer.org/Cancer/MalignantMesothelioma/DetailedGuide/malignant-mesothelioma-staging Part 5 Webpages Part 8: Tools Review of Part 5 – Treating mesothelioma • Cancer treatment is based on the cancer stage and cell type. • Chemotherapy is the use of drugs to treat cancer. • Radiation therapy uses high-energy rays to treat cancer. • There are 2 types of surgery for mesothelioma: P/D and EPP. • Primary treatment is the main treatment used to rid your body of cancer. • Neoadjuvant treatment is given before surgery; adjuvant treatment is given after surgery. Webpages American Cancer Society www.cancer.org/Cancer/MalignantMesothelioma/DetailedGuide/malignant-mesothelioma-treating-by-extent www.cancer.org/Cancer/MalignantMesothelioma/DetailedGuide/malignant-mesothelioma-treating-radiation www.cancer.org/Cancer/MalignantMesothelioma/DetailedGuide/malignant-mesothelioma-treating-chemotherapy National Cancer Institute www.cancer.gov/cancertopics/pdq/treatment/malignantmesothelioma/patient/page4 Mesothelioma Applied Research Foundation www.curemeso.org/site/c.kkLUJ7MPKtH/b.3081295/k.3C34/Pleural_Mesothelioma_Treatment.htm NCCN Guidelines for Patients®: Malignant Pleural Mesothelioma Version 1.2012 40 Part 1 • How much will the treatment cost? How can I find out how much my insurance company will cover? • How likely is it that I’ll be cancer-free after treatment? • Where will I be treated? Will I have to stay in the hospital or can I go home after each treatment? • What symptoms should I look out for while being treated for mesothelioma? • What can I do to prepare for treatment? • When will I be able to return to my normal activities? • How many mesothelioma surgeries have you done? How many of your patients have had complications? • What is the chance that my cancer will come back and/ or spread? • Is mesothelioma surgery a major part of your practice? • What should I do after I finish treatment? NCCN Guidelines for Patients®: Malignant Pleural Mesothelioma Version 1.2012 41 Part 9 Part 8 • Will my age, general health, cancer stage, or other medical conditions limit my treatment choices? Part 4 • What are the risks and benefits of each treatment? Part 5 • How soon should I start treatment? How long does treatment take? Part 6 • What are the available treatments for mesothelioma? Do I have to get treated? Part 7 Questions about treatment to ask your doctor Part 3 Part 2 Part 8: Tools Part 8: Tools Review of Part 6 – Supportive care • Supportive care is the treatment of symptoms of a disease. • Talc pleurodesis or a pleural catheter can treat pleural effusion. • Radiation therapy, chemotherapy, or pain drugs are used to treat chest pain. • Talk to your doctor about ways to quit smoking and treatment for distress. Webpages American Cancer Society www.cancer.org/Treatment/index National Cancer Institute www.cancer.gov/cancertopics/coping NCCN www.nccn.com/living-with-cancer.html CURE magazine http://media.curetoday.com/downloads/documents/pocketguide_mesothelioma.pdf NCCN Guidelines for Patients®: Malignant Pleural Mesothelioma Version 1.2012 42 Part 1 • A treatment plan can help you through cancer treatment and beyond. • It covers many issues—test results, treatments, and supportive programs. Part 2 Review of Part 7 – Accepting a treatment plan Part 3 Part 8: Tools Part 4 • You may wish to get a 2nd opinion on your treatment plan. American Cancer Society www.cancer.org/Treatment/FindingandPayingforTreatment/index NCCN Guidelines for Patients®: Malignant Pleural Mesothelioma Version 1.2012 43 Part 9 Part 8 Part 7 Part 6 National Cancer Institute www.cancer.gov/cancertopics/factsheet/Therapy/doctor-facility Part 5 Webpages Part 9: Dictionary Abdomen The belly area between the chest and pelvis. Asbestos A group of minerals made of tiny fibers that are strong, flexible, and resistant to heat. Body plethysmograph A test that measures how much air your lungs can hold and how much air is in your lungs after you exhale. Biomarker A substance found in body fluid or tissues that may be a sign of cancer. Bronchoscope A thin, long tube fitted with tools that is guided down the throat. Adjuvant treatment A treatment given after the main treatment used to cure disease. Biopsy Removal of small amounts of tissue or fluid to be tested for disease. Cancer staging Ratings of the growth and spread of tumors. Allergic reaction Symptoms caused when the body is trying to rid itself of invaders. Biphasic (mixed) subtype A subtype of pleural mesothelioma that has organized, structured cells (epithelioid) and long, spindle-shaped, disorganized cells (sarcomatoid). Cardiac stress test A test of how well your heart works during exercise. Abrams needle biopsy A biopsy with a type of needle designed to limit the amount of air that enters tissue. Amosite asbestos Straight, brittle, needle-like asbestos fibers. Amphibole asbestos A group of asbestos fibers that are straight, needle-like, and brittle. Anesthesia Loss of feeling with or without loss of wakefulness. Blood vessel A tube that circulates blood throughout the body. Board-certified A status to identify doctors who finished training in a specialized field of medicine. Artery A tube that carries blood from the heart to organs throughout the body. NCCN Guidelines for Patients®: Malignant Pleural Mesothelioma Version 1.2012 Cells The “building blocks” of tissues in the body. Chemotherapy Drugs that stop the growth process of cells in an active growth phase. Chemotherapy cycle Days of treatment followed by days of rest. Chest wall The layer of muscles and bones under the skin that covers the chest area. 44 Contrast A dye put into your body to make clearer pictures during imaging tests. Core biopsy Removal of a larger sample of tissue using a wide needle to be tested for disease. Crocidolite asbestos Straight, brittle, needle-like asbestos fibers. Diaphragm A sheet of muscles below the ribs that helps a person to breathe. Distant metastasis The spread of cancer cells from the first tumor to a far site. Electrocardiography (ECG) machine A machine that measures the electrical activity of the heart during exercise. NCCN Guidelines for Patients®: Malignant Pleural Mesothelioma Version 1.2012 45 External beam radiation therapy (EBRT) Radiation therapy received from a machine outside the body. Extrapleural pneumonectomy (EPP) Surgery that removes the affected lung and all its tissue lining, the sheet of muscles below, and sometimes the tissue lining around the heart. First-line treatment The first set of treatments given to treat a disease. Part 3 Part 2 Part 1 Diagnostic imaging specialist A person trained to read pictures of the body made by imaging tests. Erionite A mineral fiber similar to a type of straight, brittle, needle-like asbestos fiber. Part 4 Diagnosis To identify a disease. Part 5 Conformal radiation therapy Radiation beams are shaped to match the shape of the cancer using imaging scans. Epithelioid subtype The most common subtype of pleural mesothelioma; epithelioid cells are organized and structured. Part 6 Computed tomography (CT) scan A test that uses x-rays from many angles to view a body part. Cytokeratin A type of protein found on cells that line the inside and outside surfaces of the body. Endobronchial ultrasound–guided fine-needle aspiration (EBUS-FNA) Removal of tissue with a needle at the end of a long, thin tube guided down the main airway using imaging tests. Part 7 Clinical stage Rating of the extent of a tumor based on tests before treatment. CT-guided biopsy Use of pictures from a CT scan to find the right spot to remove a sample of tissue or fluid to test for disease. Part 8 Chrysotile asbestos Long, curly asbestos fibers. Part 9 Part 9: Dictionary Part 9: Dictionary 18F-fluorodeoxyglucose (FDG) A solution of fluoride and glucose (sugar) put in your body to make cancer show up better on certain imaging tests. Intensity-modulated radiation therapy (IMRT) Radiation therapy that uses small beams of different strengths based on the thickness of the tissue. General anesthesia A controlled loss of wakefulness from drugs. Lobe A clearly seen division in an organ or part of the body. Histologic subtype Grouping of cancer types based on differences in the structure and organization of cells. Local anesthesia A controlled loss of feeling in a small area of the body from drugs. Hives Itchy, swollen, and red skin caused by the body ridding itself of an invader. Hospice care Treatment only to reduce symptoms caused by cancer without treating the cancer itself. Imaging tests Tests that take pictures of the inside of the body. Inflammation Redness, heat, pain, and swelling from injury or infection. Lung An organ in the body made of airways and air sacs. Lymph vessels Tube-shaped ducts that carry lymph throughout the body. Magnetic resonance imaging (MRI) A test that uses radio waves and powerful magnets to see the shape and function of body parts. Mediastinal lymph nodes Groups of disease-fighting cells in the middle of the chest. Mediastinoscope A thin, long tube fitted with tools to work inside the chest. Lymph A clear fluid containing white blood cells. Mediastinoscopy Use of a thin tool inserted above the breastbone to do work in the middle of the chest. Lymph node sampling Removal of one group of diseasefighting cells from a cluster. Mediastinum The area of the chest between the lungs. Lymph nodes Small groups of special diseasefighting cells located throughout the body. Medical history All health events and medications taken to date. NCCN Guidelines for Patients®: Malignant Pleural Mesothelioma Version 1.2012 46 Multidisciplinary team A team that includes a number of doctors and other health care professionals who are experts in different areas of cancer treatment. Neoadjuvant treatment The treatment given before the main treatment used to cure disease. Pathologist A doctor who’s an expert in testing cells to find disease. Perfusion scanning A test that assesses blood flow in and out of the lungs. NCCN Guidelines for Patients®: Malignant Pleural Mesothelioma Version 1.2012 47 Pleura The tissue lining around the lungs. Pleural biopsy The removal of a sample of the tissue lining around the lungs to test for disease. Pleural catheter A tube that drains fluid from the chest. Pleural cavity The space between the two layers of tissue lining around the lungs. Pleural effusion Excess fluid between the two layers of tissue lining around the lungs. Part 4 Part 3 Part 2 Part 1 Parietal pleura The outer layer of the tissue lining around the lungs. Physical exam A review of the body by a health expert for signs of disease. Part 5 Metastasis The spread of cancer cells from the first tumor to another body part. Oxygen A gas in the air that the body needs to live. Peritoneum The tissue lining around the organs in the belly area. Part 6 Mesothelium A single layer of cells that makes lubricating fluid. Open biopsy A procedure that uses a surgical cut to remove samples of tissue or fluid to test for disease. Pericardium The tissue lining around the heart. Part 7 Mesothelioma A rare cancer that starts in the thin layer of tissue that covers most organs. Observation A period of testing right after treatment to check that treatment worked; or testing to monitor cancer to decide when to start treatment. Part 8 Medical oncologist A doctor who’s an expert in cancer drugs. Part 9 Part 9: Dictionary Part 9: Dictionary Pleural fluid The liquid in the space between the two layers of the tissue lining around the lungs. Pleural mesothelioma Cancer that starts in the tissue lining around the lungs. Pleural mesothelium One layer of cells in the tissue lining around the lungs that makes lubricating fluid. Pleural plaque A concentrated area of scarring in the tissue lining the lungs. Pleural thickening Widespread scarring in the tissue lining the lungs. Pleurectomy/decortication (P/D) Surgery that removes the tumor and affected part of the tissue lining the lung. Positron emission tomography (PET) A test that uses radioactive material to see the function of body parts. Primary treatment The main treatment used to rid the body of cancer. Radiation oncologist A doctor who’s an expert in treating cancer with radiation. Primary tumor The first mass of cancer cells in the body. Radiation therapy The use of radiation to treat cancer. Prognosis The course and outcome of a disease. Radiologist A doctor who’s an expert in reading imaging tests. Progression When cancer grows or spreads in the body. Radiotracer Matter with energy that is put into the body to make pictures clearer. Protocol A detailed plan of a medical study, treatment, or procedure. Recurrence The return of cancer after treatment. Proton beam therapy Radiation therapy that uses protons to treat a disease. Pulmonary function tests A set of breathing tests to test the strength of the lungs. Pulmonologist A doctor who’s an expert in treating lung diseases. NCCN Guidelines for Patients®: Malignant Pleural Mesothelioma Version 1.2012 48 Risk factor Something that increases the chance of getting a disease. Sarcomatoid subtype The type of pleural mesothelioma with random and irregular cell shapes. Second-line treatment The next treatment(s) given when first-line treatment fails. Part 1 Surgery An operation to remove or repair a part of the body. Simulation The steps needed to prepare for radiation therapy. Surgical exploration A procedure that looks inside your body to assess the extent of the cancer to see if surgery is possible. Single agent The use of one drug. Spine The bones, muscles, and other tissues along the back from the base of the skull to the tailbone. Talc pleurodesis A medical procedure that puts powder in the area between the two layers of tissue lining the lungs to seal the layers together to stop fluid buildup. Spirometry A test that uses a tube to measure how fast you breathe. NCCN Guidelines for Patients®: Malignant Pleural Mesothelioma Version 1.2012 49 Thoracoscope A thin, lighted tube with a lens and tools to view and remove samples of tissue from the chest. Thoracoscopic biopsy Use of thin tools inserted through a small cut in the chest to remove tissue to test for disease. Also called VATS biopsy. Three-dimensional conformal radiation therapy (3D-CRT) Radiation therapy that uses beams that match the shape of the tumor. Tomotherapy A type of intensity-modulated radiation therapy that takes images of the tumor and delivers radiation. Part 2 Side effect An unplanned physical or emotional response to treatment. Surgeon A doctor who’s an expert in operations to remove or repair a part of the body. Part 3 Thoracic surgeon A doctor who’s an expert in operating on organs inside the chest. Part 4 Supportive care Treatment for symptoms of a disease. Part 5 Serum mesothelin-related peptide (SMRP) A protein in cells of the mesothelium (the tissue lining of most organs in the body) that can be measured in the blood. Part 6 Thoracentesis Use of a needle inserted between the ribs to remove fluid around the lungs. Part 7 Sternum A flat bone in the center of the chest; also called the breastbone. Part 8 Serpentine asbestos A group of asbestos fibers that are long, curly, and unlikely to break. Part 9 Part 9: Dictionary Part 9: Dictionary Treatment plan A written course of action through cancer treatment and beyond. Tumor An overgrowth of cells. Ultrasound A test that uses sound waves to take pictures of the inside of the body. Video-assisted thoracoscopic surgery (VATS) Use of thin tools inserted between the ribs to do work in the chest. Visceral pleura The inner layer of tissue lining around the lungs. White blood cell A type of blood cell that fights infection. NCCN Guidelines for Patients®: Malignant Pleural Mesothelioma Version 1.2012 50 Credits NCCN aims to improve the care given to patients with cancer. NCCN staff work with experts to create helpful programs and resources for many stakeholders. Stakeholders include health care providers, patients, businesses, and others. One resource is the series of booklets for patients called the NCCN Patient Guidelines. Each booklet presents the standard of care for a type of cancer. NCCN Patient Guidelines NCCN abbreviations and acronyms Laura J. Hanisch, PsyD Medical Writer/ Patient Information Specialist The people involved in the making of the guidelines for patients and doctors are listed next, starting with NCCN staff: Dorothy A. Shead, MS Director Patient and Clinical Information Operations NCCN® National Comprehensive Cancer Network® Lacey Marlow Associate Medical Writer NCCN Patient Guidelines™ NCCN Guidelines for Patients™ NCCN Guidelines NCCN Guidelines® NCCN Clinical Practice Guidelines in Oncology® Kristina M. Gregory, RN, MSN, OCN Vice President/ Clinical Information Operations The patient booklets are based on guidelines written for doctors. These guidelines are called the NCCN Guidelines. They give a step-by-step course of care that many cancer doctors follow. Panels of experts create the NCCN Guidelines. Most of the experts are from the 21 NCCN Member Institutions. Panelists may include surgeons, radiation oncologists, medical oncologists, and patient advocates. Recommendations in the NCCN Guidelines are based on clinical trials and the experience of the panelists. NCCN Guidelines for Patients®: Malignant Pleural Mesothelioma Version 1.2012 Miranda Hughes, PhD Oncology Scientist/ Senior Medical Writer NCCN Marketing Rene Dubnanski Graphic Design Specialist 51 NCCN Panel Members for Malignant Pleural Mesothelioma David S. Ettinger, MD The Sidney Kimmel Comprehensive Cancer Center at Johns Hopkins Todd L. Demmy, MD Roswell Park Cancer Institute Ritsuko Komaki, MD The University of Texas MD Anderson Cancer Center Lee M. Krug, MD Memorial Sloan-Kettering Cancer Center Ramaswamy Govindan, MD Siteman Cancer Center at Barnes-Jewish Hospital and Washington University School of Medicine Wallace Akerley, MD Huntsman Cancer Institute at the University of Utah Frederic W. Grannis, Jr., MD City of Hope Comprehensive Cancer Center Mark G. Kris, MD Memorial Sloan-Kettering Cancer Center Hossein Borghaei, DO, MS Fox Chase Cancer Center Stefan C. Grant, MD, JD University of Alabama at Birmingham Comprehensive Cancer Center Rudy P. Lackner, MD UNMC Eppley Cancer Center at The Nebraska Medical Center Andrew C. Chang, MD University of Michigan Comprehensive Cancer Center Richard T. Cheney, MD Roswell Park Cancer Institute Lucian R. Chirieac, MD Dana-Farber/Brigham and Women’s Cancer Center Thomas A. D’Amico, MD Duke Cancer Institute Leora Horn, MD, MSc, FRCPC Vanderbilt-Ingram Cancer Center Thierry M. Jahan, MD UCSF Helen Diller Family Comprehensive Cancer Center Melissa Johnson, MD Robert H. Lurie Comprehensive Cancer Center of Northwestern University NCCN Guidelines for Patients®: Malignant Pleural Mesothelioma Version 1.2012 52 Feng-Ming (Spring) Kong, MD, PhD, MPH University of Michigan Comprehensive Cancer Center Inga T. Lennes, MD Massachusetts General Hospital Cancer Center Billy W. Loo, Jr., MD, PhD Stanford Cancer Institute Renato Martins, MD, MPH Fred Hutchinson Cancer Research Center/Seattle Cancer Care Alliance NCCN Panel Members for Malignant Pleural Mesothelioma Gregory A. Otterson, MD The Ohio State University Comprehensive Cancer Center – James Cancer Hospital and Solove Research Institute Jyoti D. Patel, MD Robert H. Lurie Comprehensive Cancer Center of Northwestern University Mary C. Pinder-Schenck, MD Moffitt Cancer Center Katherine M. Pisters, MD The University of Texas MD Anderson Cancer Center Karen Reckamp, MD, MS City of Hope Comprehensive Cancer Center Gregory J. Riely, MD, PhD Memorial Sloan-Kettering Cancer Center Theresa A. Shapiro, MD, PhD The Sidney Kimmel Comprehensive Cancer Center at Johns Hopkins Scott J. Swanson, MD Dana-Farber/Brigham and Women’s Cancer Center Kurt Tauer, MD University of Tennessee Cancer Institute Douglas E. Wood, MD Fred Hutchinson Cancer Research Center/Seattle Cancer Care Alliance Stephen C. Yang, MD The Sidney Kimmel Comprehensive Cancer Center at Johns Hopkins Eric Rohren, MD, PhD The University of Texas MD Anderson Cancer Center NCCN Guidelines for Patients®: Malignant Pleural Mesothelioma Version 1.2012 53 NCCN Member Institutions City of Hope Comprehensive Cancer Center Los Angeles, California 800.826.4673 cityofhope.org Dana-Farber/Brigham and Women’s Cancer Center Massachusetts General Hospital Cancer Center Boston, Massachusetts 800.320.0022 dfbwcc.org massgeneral.org/cancer Duke Cancer Institute Durham, North Carolina 888.275.3853 http://www.dukecancerinstitute.org Fox Chase Cancer Center Philadelphia, Pennsylvania 888.369.2427 foxchase.org Huntsman Cancer Institute at the University of Utah Salt Lake City, Utah 877.585.0303 huntsmancancer.org Fred Hutchinson Cancer Research Center/ Seattle Cancer Care Alliance Seattle, Washington 206.288.7222 • seattlecca.org 206.667.5000 • fhcrc.org The Sidney Kimmel Comprehensive Cancer Center at Johns Hopkins Baltimore, Maryland 410.955.8964 hopkinskimmelcancercenter.org Robert H. Lurie Comprehensive Cancer Center of Northwestern University Chicago, Illinois 866.587.4322 cancer.northwestern.edu Memorial Sloan-Kettering Cancer Center New York, New York 800.525.2225 mskcc.org NCCN Guidelines for Patients®: Malignant Pleural Mesothelioma Version 1.2012 54 Moffitt Cancer Center Tampa, Florida 800.456.3434 moffitt.org The Ohio State University Comprehensive Cancer Center James Cancer Hospital and Solove Research Institute Columbus, Ohio 800.293.5066 cancer.osu.edu Roswell Park Cancer Institute Buffalo, New York 877.275.7724 roswellpark.org Siteman Cancer Center at Barnes-Jewish Hospital and Washington University School of Medicine St. Louis, Missouri 800.600.3606 siteman.wustl.edu NCCN Member Institutions St. Jude Children’s Research Hospital/ University of Tennessee Cancer Institute Memphis, Tennessee 888.226.4343 • stjude.org 877.988.3627 • utcancer.org Stanford Cancer Institute Stanford, California 877.668.7535 cancer.stanfordhospital.com University of Alabama at Birmingham Comprehensive Cancer Center Birmingham, Alabama 800.822.0933 ccc.uab.edu UCSF Helen Diller Family Comprehensive Cancer Center San Francisco, California 800.888.8664 cancer.ucsf.edu University of Michigan Comprehensive Cancer Center Ann Arbor, Michigan 800.865.1125 mcancer.org UNMC Eppley Cancer Center at The Nebraska Medical Center Omaha, Nebraska 800.999.5465 unmc.edu/cancercenter The University of Texas MD Anderson Cancer Center Houston, Texas 877.632.6789 mdanderson.org Vanderbilt-Ingram Cancer Center Nashville, Tennessee 800.811.8480 vicc.org NCCN Guidelines for Patients®: Malignant Pleural Mesothelioma Version 1.2012 55 Index Anxiety 30, 33 Asbestos 9, 11, 36 Biopsy 12–14, 17 Cancer cells 7, 9, 12, 13, 16, 20, 22, 24, 25, 27 Cancer spread 7 Cancer staging 16, 19, 20, 39 Chemotherapy 22, 24, 27 Chest wall 6, 17, 19, 29 Computed tomography 11–14, 16, 17, 22, 24–26, 29 Depression 30 Gene changes 7, 9 Lymph nodes 7, 16, 17, 19, 23 Magnetic resonance imaging 16, 17 Mediastinoscopy 16, 17 Member institutions 54, 55 Mesothelium 4, 6, 7, 14 National Comprehensive Cancer Network 2, 51 Panel members 52, 53 Pleura 6, 9, 11, 13, 17, 19, 23, 29 Pleural biopsy 11, 13, 14, 17 Pleural cavity 6, 11, 12, 29 Pleural mesothelioma 7 Positron emission tomography 16, 29 Primary treatment 23 NCCN Guidelines for Patients®: Malignant Pleural Mesothelioma Version 1.2012 Radiation therapy 23–26, 29 Risk factors 9 Serum mesothelin-related peptide 11, 14 Supportive care 29, 30 Surgery 21–24 Extrapleural pneumonectomy 23, 24 Pleurectomy/decortication 23, 24 Symptoms 11, 29, 30, 33, 34 Tests 11–17, 21, 22 Diagnosis 11–14 Treatment planning 16, 17, 21, 33 Thoracentesis 11, 12 Treatment 21–27 Adjuvant 24, 25 Neoadjuvant 22 Primary 23 Treatment plan 32–34 Thoracoscopic biopsy 13, 14, 17 56 Notes ____________________________________________________________________________________ ____________________________________________________________________________________ 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____________________________________________________________________________________ ____________________________________________________________________________________ ____________________________________________________________________________________ ____________________________________________________________________________________ ____________________________________________________________________________________ ____________________________________________________________________________________ ____________________________________________________________________________________ NCCN Guidelines for Patients®: Malignant Pleural Mesothelioma Version 1.2012 58 Also available at NCCN.com! NCCN Guidelines for Patients™ Breast, Colon, Lung, Ovarian, and Prostate Cancers, Chronic Myelogenous Leukemia, Melanoma, Mesothelioma, and Multiple Myeloma COMING SO ON! Adolescent a nd Young Adult Cancers The same authoritative source referenced by physicians and other health care professionals is available for patients. To request a printed copy: [email protected] NCCN.org – For Clinicians • NCCN.com – For Patients Malignant Pleural Mesothelioma Presented with support from the national law firm of Baron & Budd 275 Commerce Drive, Suite 300, Fort Washington, PA 19034 • 215.690.0300 NCCN.org – For Clinicians • NCCN.com – For Patients NCCN.com-N-0053-1012