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NCCN Guidelines for Patients Anal Cancer NCCN GUIDELINES FOR PATIENTS® 2021 Anal Cancer Presented with support from: Available online at NCCN.org/patients Ü Anal Cancer It's easy to get lost in the cancer world Let NCCN Guidelines for Patients® be your guide 9 Step-by-step guides to the cancer care options likely to have the best results 9 Based on treatment guidelines used by health care providers worldwide 9 Designed to help you discuss cancer treatment with your doctors NCCN Guidelines for Patients® Anal Cancer, 2021 1 About National Comprehensive Cancer Network® NCCN Guidelines for Patients® are developed by the National Comprehensive Cancer Network® (NCCN®) NCCN Clinical Practice NCCN Guidelines NCCN Guidelines in Oncology for Patients (NCCN Guidelines®) 9 An alliance of leading 9 Developed by doctors from 9 Present information from the cancer centers across the NCCN cancer centers using NCCN Guidelines in an easy- United States devoted to the latest research and years to-learn format patient care, research, and of experience 9 For people with cancer and education 9 For providers of cancer care those who support them all over the world Cancer centers 9 Explain the cancer care that are part of NCCN: 9 Expert recommendations for options likely to have the NCCN.org/cancercenters cancer screening, diagnosis, best results and treatment Free online at Free online at NCCN.org/patientguidelines NCCN.org/guidelines and supported by funding from NCCN Foundation® These NCCN Guidelines for Patients are based on the NCCN Guidelines® for Anal Cancer, Version 1.2021 — February 16, 2021. © 2021 National Comprehensive Cancer Network, Inc. All rights reserved. NCCN Foundation seeks to support the millions of patients and their families NCCN Guidelines for Patients and illustrations herein may not be reproduced in affected by a cancer diagnosis by funding and distributing NCCN Guidelines for any form for any purpose without the express written permission of NCCN. No Patients. NCCN Foundation is also committed to advancing cancer treatment one, including doctors or patients, may use the NCCN Guidelines for Patients for by funding the nation’s promising doctors at the center of innovation in cancer any commercial purpose and may not claim, represent, or imply that the NCCN research. For more details and the full library of patient and caregiver resources, Guidelines for Patients that have been modified in any manner are derived visit NCCN.org/patients. from, based on, related to, or arise out of the NCCN Guidelines for Patients. The NCCN Guidelines are a work in progress that may be redefined as often National Comprehensive Cancer Network (NCCN) / NCCN Foundation as new significant data become available. NCCN makes no warranties of any 3025 Chemical Road, Suite 100 kind whatsoever regarding its content, use, or application and disclaims any Plymouth Meeting, PA 19462 responsibility for its application or use in any way. 215.690.0300 Supporters Sponsored by Anal Cancer Foundation (ACF) The Anal Cancer Foundation (ACF) is dedicated to ending anal cancer and improving the lives of those affected by it. ACF programs raise awareness, accelerate early detection, improve quality of life, and support research to find a cure. ACF is proud to partner with the NCCN Foundation to provide this comprehensive, evidence-based guide to support anal cancer patients and their loved ones. For help during any stage of your journey, please visit analcancerfoundation.org. Endorsed by HPV Alliance The HPV Alliance mission is to be a leading resource to empower individuals with knowledge about the impact of HPV infections on health and lifestyle to embolden them to take action to prevent, limit spread, and treat HPV infections to eliminate the risk of developing HPV related cancers, like anal cancer. HPVAlliance.org With generous support from • Patrick Abbs • Kristina Gregory • Alan Venook, MD To make a gift or learn more, please visit NCCNFoundation.org/donate or e-mail [email protected]. NCCN Guidelines for Patients® Anal Cancer, 2021 3 NCCN Guidelines for Patients® Anal Cancer, 2021 4 Anal Cancer Contents 6 Anal cancer basics 17 Treatment guide 32 Survivorship 39 Making treatment decisions 52 Words to know 53 NCCN Contributors 54 NCCN Cancer Centers 56 Index NCCN Guidelines for Patients® Anal Cancer, 2021 5 1 Anal cancer basics 7 The anus 9 Risk factors 10 Diagnosis and treatment planning 13 Staging 15 Fertility and family planning 16 Key points NCCN Guidelines for Patients® Anal Cancer, 2021 6 1 Anal cancer basics The anus Anal cancer is a rare cancer of the The anus digestive system. Anal cancer treatment should involve a team of specialists The anus is the opening through which feces with expertise in digestive health, (also called stool) exit the body. It is located chemotherapy, surgery, radiation, and at the very end of the large intestine within imaging. Everyone with anal cancer is the pelvis. The pelvis is the area of the body encouraged to consider enrolling in a between the hip bones, below the abdomen. Other organs in the pelvis include the bladder, clinical trial for treatment. rectum, vagina, and prostate. The large intestine is a long, tube-shaped organ in the Anal cancer most often starts in squamous digestive system. cells. Cancer that forms in squamous cells is known as squamous cell carcinoma. After being swallowed, food passes through Squamous cells are found in the tissues that the esophagus and into the stomach, where it form the surface of the skin and in other areas is turned into a liquid. From the stomach, food of the body. Anal squamous cell carcinoma is enters the small intestine. Here, food is broken the focus of this patient guide. Other rare types down into very small parts to allow nutrients to not covered here include anal adenocarcinoma be absorbed into the bloodstream. and anal melanoma. NCCN Guidelines for Patients® are available on rectal cancer and Partly digested food then moves into the large melanoma. intestine. The colon is the first and longest The anus The anus is the opening through which feces exit the body. Anal cancer starts in either the anal canal or the perianal region. The anal canal connects the rectum and the anal opening. The perianal region includes the anal opening and the skin directly surrounding it. NCCN Guidelines for Patients® Anal Cancer, 2021 7 1 Anal cancer basics The anus section of the large intestine. In the colon, water is absorbed from unused food, turning it into stool. Stool is then held in the last several inches of the large intestine, called the rectum, until it exits the body through the anus. Because anal cancer is rare and Anal cancer starts in either the anal canal or stigmatized, it is harder to find the perianal region. The anal canal connects the rectum and the anal opening. It is about others who have been through it. 1 and a half inches (4 centimeters) long. The “Get yourself a mentor, and when anal canal is surrounded by muscles (anal you recover, pay it forward by sphincters) that relax to allow stool to leave the body through the anus. The perianal mentoring someone else. region includes the anal opening and the skin directly surrounding it. In some cases, an anal tumor can involve both the anal canal and the – Michael perianal region. Anal cancer survivor NCCN Guidelines for Patients® Anal Cancer, 2021 8 1 Anal cancer basics Risk factors Risk factors which can make it harder for the body to clear HPV infection. A risk factor is something that increases the risk of developing a disease. Some people Infection with human immunodeficiency with no known risk factors get anal cancer. virus (HIV) And, some people with risk factors never get Taking anti-rejection medicines anal cancer. Risk factors for anal cancer are (immunosuppressants) after an organ described next. transplant A history of cervical, vulvar, or vaginal Human papillomavirus infection cancer or precancer The risk factor most strongly linked with anal cancer is long-term infection with human A history of anal precancer papillomavirus (HPV). HPV is a sexually A history of blood cancer such as transmitted virus. HPV is extremely common. lymphoma (Hodgkin or non-Hodgkin), It affects almost everyone who has ever been chronic lymphocytic leukemia, or multiple sexually active. Most people are unaware that myeloma they are–or ever were–infected. Certain autoimmune disorders, such as In most people, the immune system gets Crohn’s disease rid of (“clears”) HPV from the body. In some Smoking people, however, the virus causes long-term cell changes that develop into cancer. The A history of anal sex or sexually progression to cancer often occurs decades transmitted infection after the initial infection. Doctors are still learning why one person gets anal cancer and Risk reduction another does not. Cancers caused by HPV Anal cancer starts as areas of abnormal include anal, cervical, head and neck, penile, cells, called lesions, in or near the anus. vaginal, and vulvar cancers. These pre-cancerous lesions are known as anal dysplasia. Identifying and treating anal There are more than 100 types (called strains) dysplasia may prevent anal cancer. of HPV. Infection with some strains is more likely to lead to anal cancer and other related Other names for anal dysplasia include: cancers. High-risk forms of HPV include HPV- 16 and HPV-18. HPV infection can also cause Low-grade squamous intraepithelial lesion abnormal skin growths, called warts, to form (LSIL) (mild dysplasia) on the anus or genitals. High-grade squamous intraepithelial lesion (HSIL) (moderate or severe Other risk factors dysplasia) The health- and lifestyle-related factors listed below can also increase the risk of anal Anal intraepithelial neoplasia (AIN) cancer. Some of these factors lead to a higher Stage 0 anal cancer risk because they weaken the immune system, NCCN Guidelines for Patients® Anal Cancer, 2021 9 1 Anal cancer basics Diagnosis and treatment planning The HPV vaccine Digital rectal exam A vaccine that protects against 9 different The digital rectal exam (DRE) is an important strains of HPV, including the highest-risk exam for diagnosis, staging, and follow-up.
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