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American Joint Committee on Cancer Executive Office 633 North Saint Clair Street Chicago, IL 60611-3211 Editors Stephen B. Edge, M . D ., F . A . C . S . Roswell Park Cancer Institute Buffalo, NY, USA David R. Byrd, M . D ., F . A . C . S . University of Washington School of Medicine Seattle, WA, USA Carolyn C. Compton, M . D ., P h. D . National Cancer Institute Bethesda, MD, USA April G. Fritz, R . H . I . T ., C . T . R . A. Fritz and Associates Reno, NV, USA Frederick L. Greene, M . D ., F . A . C . S . Carolinas Medical Center Charlotte, NC, USA Andy Trotti, III, M . D . H. Lee Moffitt Cancer Center Tampa, FL, USA ISBN 978-0-387-88442-4 e-ISBN 978-0-387-88443-1 Springer New York Dordrecht Heidelberg London Library of Congress Control Number: 2009930461 First to Fifth Editions of the AJCC Cancer Staging Manual, and the AJCC Cancer Staging Handbook, published by Lippincott Raven Publishers, Philadelphia. PA. Sixth Edition of the AJCC Cancer Staging Handbook, published by Springer-Verlag, New York, NY. Seventh Edition © 2010 American Joint Committee on Cancer. All rights reserved. The AJCC Cancer Staging Handbook is the Official Publication of the American Joint Committee on Cancer. This book may not be translated or copied in whole or in part without the written permission of the publisher (Springer Science+Business Media LLC, 233 Spring Street, New York, NY 10013, USA), or the copyright holder, except for brief excerpts in connection with reviews or scholarly analysis. Use in connection with any form of information storage and retrieval, electronic adaptation, computer software, or by similar or dissimilar methodology now known or hereafter developed is forbidden. Materials appearing in this book prepared by individuals as part of their official duties as U.S. Government employees are not covered by the above-mentioned copyright. The use in this publication of trade names, trademarks, service marks, and similar terms, even if they are not identified as such, is not to be taken as an expression of opinion as to whether or not they are subject to proprietary rights. While the advice and information in this book are believed to be true and accurate at the date of going to press, neither the authors nor the editors nor the publisher nor the AJCC can accept any legal responsibility for any errors or omissions that may be made. The publisher makes no warranty, express or implied, with respect to the material contained therein. Printed on acid-free paper (Corrected at 5 th printing 2010) Springer is part of Springer Science+Business Media (www.springer.com)
5 Larynx (Nonepithelial tumors such as those of lymphoid tissue, soft tissue, bone, and cartilage are not included) At-A-Glance SUMMARY OF CHANGES T4 lesions have been divided into T4a (moderately advanced local disease) and T4b (very advanced local disease), leading to the stratification of Stage IV into Stage IVA (moderately advanced local/regional disease), Stage IVB (very advanced local/regional disease), and Stage IVC (distant metastatic disease) ICD-O-3 TOPOGRAPHY CODES C10.1 Anterior (lingual) surface of epiglottis C32.0 Glottis C32.1 Supraglottis (laryngeal surface) C32.2 Subglottis C32.8* Overlapping lesion of larynx C32.9* Larynx, NOS * Stage by location of tumor bulk or epicenter ICD-O-3 HISTOLOGY CODE RANGES 8000–8576, 8940–8950, 8980–8981 5 ANATOMIC STAGE/PROGNOSTIC GROUPS Stage 0 Stage I Stage II Stage III Tis T1 T2 T3 T1 T2 T3 T4a T4a T1 T2 T3 T4a T4b Any T Any T N0 N0 N0 N0 N1 N1 N1 N0 N1 N2 N2 N2 N2 Any N N3 Any N M0 M0 M0 M0 M0 M0 M0 M0 M0 M0 M0 M0 M0 M0 M0 M1 Stage IVA Stage IVB Stage IVC ANATOMY Primary Site. The following anatomic definition of the larynx allows classification of carcinomas arising in the encompassed mucous membranes but excludes cancers arising on the lateral or posterior pharyngeal wall, pyriform fossa, postcricoid area, or base of tongue. Larynx 81
9 Mucosal Melanoma of the Head and Neck At-A-Glance SUMMARY OF CHANGES This is a new chapter for classification of this rare tumor ICD-O-3 TOPOGRAPHY CODES For a complete description of codes, refer to the appropriate anatomic site chapter based on the location of the mucosal melanoma (see Chapters 3–6) ANATOMIC STAGE/PROGNOSTIC GROUPS Stage III Stage IVA Stage IVB Stage IVC T3 T4a T3–T4a T4b Any T N0 N0 N1 Any N Any N M0 M0 M0 M0 M1 Additionally, mucosal melanomas are staged for the following topography codes; however, no staging exists for nonmucosal melanoma in the same anatomic site: C14.0 Pharynx, NOS C14.2 Waldeyer’s ring C14.8 Overlapping lesion of lip, oral cavity and pharynx The following topography codes are excluded: C07.9 Parotid gland C30.1 Middle ear C08.0 Submandibular gland C73.9 Thyroid C08.1 Sublingual gland C08.8 Overlapping lesion of major ICD-O-3 HISTOLOGY CODE RANGES salivary glands C08.9 Major salivary glands, NOS 8720–8790 9 Mucosal Melanoma of the Head and Neck 123
ANATOMIC STAGE/PROGNOSTIC GROUPS CONTINUED C16.2 Adenocarcinoma Stage T N 0 IA IB IIA IIB IIIA Tis (HGD) N0 T1 T1 T2 T2 T3 T1–2 T1–2 T3 T4a T3 T4a T4b Any Any N0 N0 N0 N0 N0 N1 N2 N1 N0 N2 Body of stomach, proximal 5 cm only* M Grade M0 1, X M0 1–2, X M0 3 M0 1–2, X M0 3 M0 Any M0 Any M0 Any M0 Any M0 Any M0 Any *Note: If gastric tumor extends to or above esophagogastric junction. ICD-O-3 HISTOLOGY CODE RANGES 8000–8576, 8940–8950, 8980–8981 (C15 only) 8000–8152, 8154–8231, 8243–8245, 8247–8248, 8250–8576, 8940–8950, 8980–8981 (C16 only) IIIB IIIC N1–2 M0 Any Any M0 Any N3 M0 Any Any M1 Any IV INTRODUCTION Previous stage groupings of esophageal cancer were based on a simple, orderly arrangement of increasing pathologic anatomic T, then N, and then M classifications. In contrast, this revision is data driven, based on a risk- adjusted random-survival-forest analysis of worldwide data. The previous system was neither consistent with these data nor biologically plausible. Some explanations for the discrepancy relate to the interplay among T, N, and M, histopathologic type, biologic activity of the tumor (histologic grade), and location. The unique lymphatic anatomy of the esophagus links N to T, permit- ting lymph node metastases from superficial cancers (pT1); this renders prognosis similar to that of more advanced (higher pT) N0 cancers. Simi- larly, advanced cancers (higher pT) with a few positive nodes may have a similar prognosis to those of less advanced cancers (lower pT) with more positive nodes. Biologic activity of the cancer, reflected by histologic grade (G), modulates stage such that prognosis of well-differentiated (G1) higher- pT cancers is similar to that of less well-differentiated (G2–G4) lower-pT cancers. Previous staging recommendations ignored histopathologic type, but availability of data on a large mixture of adenocarcinoma and squamous cell carcinomas from around the world has permitted assessing the association of histopathologic type with survival. Although at first glance these multiple trade-offs seem to create a less orderly arrangement of cancer classifications within and among stage 130 American Joint Committee on Cancer • 2010
ANATOMIC STAGE/PROGNOSTIC GROUPS CONTINUED C16.6 Stage IIIB T4b T4b T4a T3 T4b T4b T4a Any T N0 N1 N2 N3 N2 N3 N3 Any N M0 M0 M0 M0 M0 M0 M0 M1 C16.8 C16.9 Greater curvature of stomach, NOS Overlapping lesion of stomach Stomach, NOS Stage IIIC Stage IV ICD-O-3 HISTOLOGY CODE RANGES 8000–8152, 8154–8231, 8243–8245, 8247–8248, 8250–8576, 8940–8950, 8980–8990 INTRODUCTION Gastric cancer remains the fourth most common cancer worldwide and the second leading cause of cancer deaths (700,000 deaths annually worldwide). The highest rates of this disease continue to be in areas of Asia and Eastern Europe. Although gastric adenocarcinoma has declined significantly in the USA over the past 70 years, during the early twenty-first century an estimated 22,000 patients develop the disease each year, and of these patients, 13,000 will die, mainly because of nodal and metastatic disease present at the time of initial diagnosis. Trends in survival rates from the 1970s to the 1990s have unfortunately shown very little improvement. During the 1990s, 20% of gastric carcinoma cases were diagnosed while localized to the gastric wall, whereas 30% had evidence of regional nodal disease. Disease resulting from metastasis to other solid organs within the abdomen, as well as to extraab- dominal sites, represents 35% of all cases. Although overall 5-year survival is approximately 15–20%, the 5-year survival is approximately 55% when disease is localized to the stomach (Figure 11.1). The involvement of regional nodes reduces the 5-year survival to approximately 20%. A notable shift in the site of gastric cancer reflects a proportionate increase in disease of the proximal stomach over the past several decades. Previously, there was a predominance of distal gastric cancers presenting as mass lesions or ulceration. Although other malignancies occur in the stomach, approximately 90% of all gastric neoplasms are adenocarcino- mas. Tumors of the esophagogastric junction (EGJ) may be difficult to stage as either a gastric or an esophageal primary, especially in view of the increased incidence of adenocarcinoma in the esophagus that presumably results from acid reflux disease. ANATOMY Primary Site. The stomach is the first division of the abdominal portion of the alimentary tract, beginning at the esophagogastric junction and extend- ing to the pylorus. The proximal stomach is located immediately below the diaphragm and is termed the cardia. The remaining portions are the 146 American Joint Committee on Cancer • 2010
12 Small Intestine (Lymphomas, carcinoid tumors, and visceral sarcomas are not included) At-A-Glance SUMMARY OF CHANGES T1 lesions have been divided into T1a (invasion of lamina propria) and T1b (invasion of submucosa) to facilitate comparison with tumors of other gastrointestinal sites Stage II has been subdivided into Stage IIA and Stage IIB The N1 category has been changed to N1 (1–3 positive lymph nodes) and N2 (four or more positive lymph nodes), leading to the division of Stage III into Stage IIIA and Stage IIIB ICD-O-3 TOPOGRAPHY CODES C17.0 Duodenum C17.1 Jejunum C17.2 Ileum C17.8 Overlapping lesion of small intestine C17.9 Small intestine, NOS ICD-O-3 HISTOLOGY CODE RANGES 8000–8152, 8154–8231, 8243–8245, 8247–8248, 8250–8576, 8940–8950, 8980–8981 ANATOMIC STAGE/PROGNOSTIC GROUPS Stage 0 Stage I Stage IIA Stage IIB Stage IIIA Stage IIIB Stage IV Tis T1 T2 T3 T4 Any T Any T Any T N0 N0 N0 N0 N0 N1 N2 Any N M0 M0 M0 M0 M0 M0 M0 M1 INTRODUCTION Although the small intestine accounts for one of the largest surface areas in the human body, it is one of the least common cancer sites in the diges- tive system, accounting for less than 2% of all malignant tumors of the gastrointestinal tract. A variety of tumors occur in the small intestine, with approximately 25–50% of the primary malignant tumors being adeno- carcinomas, depending upon the population surveyed. At the beginning of the twenty-first century, approximately 5,600 new cases of cancer involving the small intestine are seen annually in the USA. The 1,100 deaths predicted Small Intestine 12 153
ANATOMIC STAGE/PROGNOSTIC GROUPS Stage T 0 I IIA IIB IIC Tis T1 T2 T3 T4a T4b N N0 N0 N0 N0 N0 N0 M Dukes* MAC* A B1 B2 B2 B3 C1 C1 C2 C1/C2 C1 C2 C2 C3 M0 – M0 A M0 A M0 B M0 B M0 B IIIA T1–T2 N1/N1c M0 C T1 N2a M0 C IIIB T3–T4a N1/N1c M0 C T2–T3 N2a M0 C T1–T2 N2b M0 C IIIC T4a N2a M0 C T3–T4a N2b M0 C T4b N1–N2 M0 C IVA Any T Any N Any N M1a – M1b – IVB Any T ICD-O-3 TOPOGRAPHY CODES C18.0 Cecum C18.2 Ascending colon C18.3 Hepatic flexure of colon C18.4 Transverse colon C18.5 Splenic flexure of colon C18.6 Descending colon C18.7 Sigmoid colon C18.8 Overlapping lesion of colon C18.9 Colon, NOS C19.9 Rectosigmoid junction C20.9 Rectum, NOS ICD-O-3 HISTOLOGY CODE RANGES 8000–8152, 8154–8231, 8243–8245, 8247–8248, 8250–8576, 8940–8950, 8980–8981 Note : cTNM is the clinical classification, pTNM is the pathologic classification. The y prefix is used for those cancers that are classified after neoadjuvant pretreat- ment (e.g., ypTNM). Patients who have a complete pathologic response are ypT0N0cM0 that may be similar to Stage Group 0 or I. The r prefix is to be used for those cancers that have recurred after a disease-free interval (rTNM). *Dukes B is a composite of better (T3 N0 M0) and worse (T4 N0 M0) prognostic groups, as is Dukes C (Any TN1 M0 and Any T N2 M0). MAC is the modi- fied Astler-Coller classification. INTRODUCTION The TNM classification for carcinomas of the colon and rectum provides more detail than other staging systems. Compatible with the Dukes’ sys- tem, the TNM adds greater precision in the identification of prognostic subgroups. TNM staging is based on the depth of tumor invasion into or beyond the wall of the colorectum (T), invasion of or adherence to adjacent organs or structures (T), the number of regional lymph nodes involved (N), and the presence or absence of distant metastasis (M). The TNM classification applies to both clinical and pathologic staging. Most cancers of the colon and many cancers of the rectum are staged after pathologic examination of a resected specimen. However, patients with 174 American Joint Committee on Cancer • 2010
specimens of ascending colon, descending colon, or upper rectum is only partially peritonealized, and the demarcation between the peritonealized surface and the nonperitonealized surface (corresponding to the CRM) of such specimens is not always easily appreciated on pathologic examination. Therefore, the surgeon is encouraged to mark the peritoneal reflection and/ or the area of deepest tumor penetration adjacent to a nonperitonealized surface with a clip or suture so that the pathologist may accurately identify and evaluate the CRM. For mid and distal rectal cancers (subperitoneal location), the entire sur- face of the resection specimen corresponds to a CRM (anterior, posterior, medial, lateral). For proximal rectal or retroperitoneal colon cancers (ascend- ing, descending, possibly cecum), surgically dissected margins will include those that lie in a retroperitoneal or subperitoneal location as described above (Figure 14.3). For segments of the colon that are entirely covered by a visceral peritoneum (transverse, sigmoid, possibly cecum), the only speci- men margin that is surgically dissected is the mesenteric margin, unless the cancer is adherent to or invading an adjacent organ or structure. Therefore, for cancers of the cecum, transverse or sigmoid colon that extends to the cut edge of the mesentery, assignment of a positive CRM is appropriate. For rectal cancer, the quality of the surgical technique is likely a key factor in the success of surgical outcomes relative to local recurrence and possibly long-term survival. Numerous nonrandomized studies have dem- onstrated that total mesorectal excision (TME) with adequate surgical clearance around the penetrating edge of the tumor decreases the rate of local relapse. The TME technique entails precise sharp dissection within the areolar plane of loose connective tissue outside (lateral to) the visceral mesorectal fascia in order to remove the rectum. With this approach, all mesorectal soft tissues encasing the rectum, which includes the mesentery 14 FIGURE 14.3. Circumferential resection margin. Colon and Rectum 193
15 FIGURE 15.1. Anatomic subsites of the anal canal. Determination of the anatomic site of origin of carcinomas that over- lap the anorectal junction may be problematic. For staging purposes, such tumors should be classified as rectal cancers if their epicenter is located more than 2 cm proximal to the dentate line or proximal to the anorectal ring on digital examination and as anal canal cancers if their epicenter is 2 cm or less from the dentate line. For rectal cancers that extend beyond the dentate line, as for anal canal cancers, the superficial inguinal lymph nodes are among the regional nodal groups at risk of metastatic spread and included in cN/pN analysis (see later). Regional Lymph Nodes. Lymphatic drainage and nodal involvement of anal cancers depend on the location of the primary tumor. Tumors above the dentate line spread primarily to the anorectal, perirectal, and paraverte- bral nodes, whereas tumors below the dentate line spread primarily to the superficial inguinal nodes. The regional lymph nodes are as follows (Figure 15.2): Perirectal Anorectal Perirectal Lateral sacral Internal iliac (hypogastric) Inguinal Superficial All other nodal groups represent sites of distant metastasis. Metastatic Sites. Cancers of the anus may metastasize to any organs, but the liver and lungs are the distal organs that are most frequently involved. Involvement of the abdominal cavity is not unusual. Anus 209
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