our Pleaseonline completesurvey at NCCN.org/patients/survey NCCN GUIDELINES FOR PATIENTS® Version 1.2016 Malignant Pleural Mesothelioma Presented with support from: Available online at NCCN.org/patients Ü Malignant Pleural Mesothelioma that you have cancer LEARNING can be overwhelming. The goal of this book is to help you get the best care. It explains which tests and treatments are recommended by experts for malignant pleural mesothelioma. The National Comprehensive Cancer Network® (NCCN®) is a not-for-profit alliance of 27 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 patient book is based on the guidelines written for doctors. This book focuses on the treatment of mesothelioma. Key points of the book are summarized in the NCCN Quick Guide™ series Malignant Pleural Mesothelioma. NCCN also offers patient books on breast cancer, lung cancer, melanoma, and many other cancer types. Visit NCCN.org/patients for the full library of patient books as well as other patient and caregiver resources. NCCN Guidelines for Patients®: Malignant Pleural Mesothelioma, Version 1.2016 1 About These patient guides for cancer care are produced by the National Comprehensive Cancer Network® (NCCN®). The mission of NCCN is to improve cancer care so people can live better lives. At the core of NCCN are the NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®). NCCN Guidelines® contain information to help health care workers plan the best cancer care. They list options for cancer care that are most likely to have the best results. The NCCN Guidelines for Patients® present the information from the NCCN Guidelines in an easy-to-learn format. Panels of experts create the NCCN Guidelines. Most of the experts are from 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. The NCCN Guidelines are updated at least once a year. When funded, the patient books are updated to reflect the most recent version of the NCCN Guidelines for doctors. For more information about the NCCN Guidelines, visit NCCN.org/clinical.asp. Dorothy A. Shead, MS Alycia Corrigan Susan Kidney Director, Patient and Medical Writer Graphic Design Clinical Information Specialist Operations Rachael Clarke Guidelines Data and Kimberly Williams Laura J. Hanisch, PsyD Layout Coordinator Graphic Design and Medical Writer/Patient Production Specialist Information Specialist NCCN Foundation was founded by NCCN to raise funds for patient education based on the NCCN Guidelines. NCCN Foundation offers guidance to people with cancer and their caregivers at every step of their cancer journey. This is done by sharing key information from the world’s leading cancer experts. This information can be found in a library of NCCN Guidelines for Patients® and other patient education resources. NCCN Foundation is also committed to advancing cancer treatment by funding the nation’s promising doctors at the center of cancer research, education, and progress of cancer therapies. For more information about NCCN Foundation, visit NCCNFoundation.org. © 2016 National Comprehensive Cancer Network, Inc. All rights reserved. NCCN Guidelines for Patients® and illustrations herein may not be reproduced in any form for any purpose without the express written permission of NCCN. National Comprehensive Cancer Network (NCCN) • 275 Commerce Drive, Suite 300 • Fort Washington, PA 19034 • 215.690.0300 NCCN Guidelines for Patients®: Malignant Pleural Mesothelioma, Version 1.2016 2 Supporters Endorsed and sponsored in part by Mesothelioma Applied® Research Foundation Pursuant to its mission to eradicate mesothelioma, the Mesothelioma Applied® Research Foundation supports all efforts to help patients find, understand, and undergo critical and life-saving treatments. We endorse this guide as a valuable and empowering resource for patients. curemeso.org NCCN Guidelines for Patients®: Malignant Pleural Mesothelioma, Version 1.2016 3 NCCN Guidelines for Patients®: Malignant Pleural Mesothelioma, Version 1.2016 4 Malignant Pleural Mesothelioma Contents 6 How to use this book 29 Part 5 Supportive care 7 Part 1 Presents the recommended treatments About mesothelioma for the physical and emotional challenges Explains malignant pleural mesothelioma of mesothelioma. and the risk factors for this cancer. 33 Part 6 12 Part 2 Making treatment decisions Testing for mesothelioma Offers information to help you and your Explains when you should be tested and doctors create a good treatment plan for the tests used. you. 17 Part 3 39 Glossary Treatment planning Dictionary Describes the tests used to plan Acronyms treatment based on how far the cancer has spread. 48 NCCN Panel Members 21 Part 4 49 NCCN Member Institutions Treatment guide Presents the recommended treatment 50 Index options for malignant pleural mesothelioma. NCCN Guidelines for Patients®: Malignant Pleural Mesothelioma, Version 1.2016 5 How to use this book Who should read this book? Help! What do the words mean? Mesothelioma is a rare cancer of the mesothelium— a layer of cells that makes fluid. Mesothelioma In this book, many medical words are included. can start in the lining of the abdomen (peritoneal These are words you will likely hear from your mesothelioma), heart (pericardial mesothelioma), treatment team. Most of these words may be new to testicles (testicular mesothelioma), or lungs (pleural you, and it may be a lot to learn. mesothelioma). It is a rare type of cancer. This book focuses on mesothelioma that starts in the lining of Don’t be discouraged as you read. Keep reading the lungs. Patients and those who support them may and review the information. Feel free to ask your find this book helpful. treatment team to explain a word or phrase that you do not understand. Words that you may not know are defined in the text Does the whole book apply or in the Dictionary. Acronyms are also defined when to me? first used and in the Glossary. Acronyms are short words formed from the first letters of several words. This book includes information for many situations. One example is CT for computed tomography. Your medical team can help. They can point out what parts of the book apply to you. They can also give you more information. As you read through this book, you may find it helpful to make a list of questions to ask your doctors. The recommendations in this book are based on science and the experience of NCCN experts. However, these recommendations may not be right for you. Your doctors may suggest other tests or treatments based on your health and other factors. If other suggestions are given, feel free to ask your treatment team questions. NCCN Guidelines for Patients®: Malignant Pleural Mesothelioma, Version 1.2016 6 1 About mesothelioma 8 What is a mesothelium? 8 How does mesothelioma start? 10 What are the risk factors? 11 Review NCCN Guidelines for Patients®: Malignant Pleural Mesothelioma, Version 1.2016 7 What is a mesothelium? About mesothelioma 1 How does mesothelioma start? In Part 1, you will learn about cancer How does mesothelioma start? cells and where mesothelioma grows in the body. 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 What is a mesothelium? than normal cells. The lungs are covered by a double layer of thin Normal cells grow and then divide to form new cells tissue called the pleura. The pleura protects and as the body needs them. When normal cells grow old cushions the lungs. It has an inner layer called the or get damaged, they die. Cancer cells don’t do this. visceral pleura. It is directly attached to the lungs and Cancer cells make new cells that aren’t needed and extends down into the spaces between the lobes don’t die quickly when old or damaged. Over time, of the lungs. The outer layer is calleed the parietal the cancer cells grow and divide enough to form a pleura. It lines the chest wall and has contact with mass called the primary tumor. If the primary tumor many body parts in the chest. See Figure 1. isn’t treated, it can grow and invade nearby tissues and organs, such as the lungs. The chest wall is the layer of muscle and bones under the skin in the chest area. The pleura is made Unlike normal cells, cancer cells can spread and of mesothelium and connective tissue. form tumors in other parts of the body. Cancer that has spread is called a metastasis. Cancer cells often Mesothelium is a single layer of cells that spread through lymph or blood. Lymph is a clear makes lubricating fluid and lines most organs fluid that gives cells water and food. It also has white inside the body. blood cells that fight germs. Lymph nodes are groups of disease-fighting cells that filter lymph and remove Connective tissue supports the mesothelium the germs. Lymph travels throughout the body in and supplies it with blood. Connective tissue vessels like blood does. As shown in Figure 2, attaches the pleura to other body parts. lymph vessels and nodes are found everywhere in the body. There is space between the visceral and parietal pleura called the pleural cavity. It is filled with a small Malignant pleural mesothelioma is the medical name amount of pleural fluid made by the mesothelium. for cancer that starts in the mesothelium of the lungs. Pleural fluid acts as a lubricant. It helps the two It is also simply called mesothelioma. It isn’t lung pleural layers slide against each other during cancer.