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Originally published as JCO Early Release 10.1200/JCO.2008.20.0014 on November 17 2008 © 2008 American Society of Clinical Oncology.
In Reply:
Department of Gastrointestinal Medical Oncology, The University of Texas M. D. Anderson Cancer Center, Houston, Texas In the accompanying correspondence, Dr Scherübl made several observations regarding our article.1 A number of these points warrant our attention. Dr Scherübl correctly notes that our survival analyses reflect overall survival and not disease-specific survival. While the Surveillance, Epidemiology, and End Results (SEER) program database provides data on cause of death based on International Classification of Diseases (ICD), we chose to perform overall survival analyses for several reasons. First, accurate assessment of cause of death is needed for disease-specific survival analyses. While it is possible to reliably ascertain the cause of death by meticulous review of medical records in a small retrospective case series, this is not possible with population-based registries. In the case of neuroendocrine tumors (NETs), this is further complicated by the lack of specific ICD-9 codes for the majority of NETs. Until now, ICD-9-CM, the official system of assigning codes to diagnoses and procedures associated with medical resource utilization in the United States, only contained discrete codes for islet cell carcinoma (157.4) and hormonal syndrome due to carcinoid tumors (carcinoid syndrome, 259.2). Other NETs such as small bowel or rectal carcinoids were coded as carcinomas of respective primary sites. Thus, it would have been impossible to distinguish a rectal carcinoid from a rectal adenocarcinoma based on ICD-9-CM codes. While it is possible to tease out the NET diagnoses from the SEER database using International Classification of Diseases for Oncology, third edition, codes, it cannot be used to determine cause of death. ICD-9-CM codes are also the means by which we communicate diagnoses between health care providers and third party payors. As we enter the age of targeted therapy for NETs and develop effective antineoplastic therapy beyond symptom control,2-4 we will also need NET-specific ICD-9-CM codes to offer our patients appropriate therapy. To this end, we approached the Center for Disease Control and the Centers for Medicare and Medicaid Services in 2007 and applied for a disease-specific set of ICD-9-CM codes for NETs, which came into effect on October 1, 2008 (Table 1). It is hoped that these new codes, in time, would also provide the means to perform more detailed analyses from population-based registries such as SEER.
Dr Scherübl also makes a number of observations about the prognosis of patients with rectal carcinoids. We agree that there is tremendous heterogeneity in the prognosis of patients with carcinoids by primary site.1 The vast majority of rectal carcinoids (92%) are localized at diagnosis and have a good prognosis (5-year survival rate, 90%). Those that are metastatic at diagnosis (5%) have inferior 5-year survival rates (24%) compared with carcinoids of most other primary sites. Nonetheless, the prognosis of patients with rectal carcinoids is better than that of patients with rectal cancer in general. For example, during the period from 1987 to 2004, the overall 5-year survival rate for rectal carcinoid was higher (88% v 51%) than those with other rectal malignancies (excluding rectal NETs).5,6 The 5-year survival rates were also better for patients with carcinoids within each SEER stage group (localized, 88% v 69%; regional, 62% v 49%; distant, 24% v 7%).1,6
Dr Scherübl argues that if lymph node dissection is performed, even 6-mm, well-differentiated, "benign-appearing" rectal carcinoid with normal-appearing regional lymph nodes on imaging may be found to have malignant nodal involvement. While we agree that, rarely, even a small rectal carcinoid may spread, we would caution against recommending extensive surgery for well-differentiated rectal NETs that are less than 1 cm in absence of a prospective randomized study demonstrating benefit. Data from two large autopsy series suggests that small carcinoids are found in 0.65%7 to 1.22%8 of cases, while data from population-based registries show the prevalence rate for clinically significant NETs is far less.1 This suggests that many people have small benign carcinoids that are destined to remain clinically silent during their lives. Given the excellent prognosis of patients with small rectal carcinoids, the surgical morbidity and mortality associated with open resection (low anterior resection or abdominoperineal resection) is not justified for most patients. We support the National Comprehensive Cancer Network guideline recommendation for endoscopic or transanal resection of rectal carcinoid Data from population-based registries suggests that NETs consist of a group of related diseases that vary in prognosis by primary site. Optimal management requires a multidisciplinary approach. Ongoing randomized phase III studies with mTOR inhibitor, everolimus, and the vascular endothelial growth factor inhibitors bevacizumab and sunitinib may lead to further advances in therapy for NETs. AUTHOR's DISCLOSURES OF POTENTIAL CONFLICTS OF INTEREST The author(s) indicated no potential conflicts of interest.
NOTES published online ahead of print at www.jco.org on November 17, 2008. REFERENCES
1. Yao JC, Hassan M, Phan A, et al: One hundred years after "carcinoid": Epidemiology of and prognostic factors for neuroendocrine tumors in 35,825 cases in the United States. J Clin Oncol 26:3063-3072, 2008 2. Yao JC, Phan A, Hoff PM, et al: Targeting vascular endothelial growth factor in advanced carcinoid tumor: A random assignment phase II study of depot octreotide with bevacizumab and pegylated interferon alpha-2b. J Clin Oncol 26:1316-1323, 2008 3. Yao JC, Phan AT, Chang DZ, et al: Efficacy of RAD001 (everolimus) and octreotide LAR in advanced low- to intermediate-grade neuroendocrine tumors: Results of a phase II study. J Clin Oncol 26:4311-4318, 2008 4. Yao JC: Molecular targeted therapy for carcinoid and islet-cell carcinoma. Best Pract Res Clin Endocrinol Metab 21:163-172, 2007[CrossRef][Medline] 5. Surveillance, Epidemiology, and End Results (SEER) Program: SEER*Stat Database: SEER 17 Regs Nov 2005 sub (1973-2003), Bethesda, MD, National Cancer Institute, 2006 6. National Cancer Institute Surveillance Research Program: SEER*Stat software. Version 6.2.4, 2006 7. Moertel CG, Sauer WG, Docherty MB, et al: Life history of the carcinoid tumor of the small intestine. Cancer 14:291-293, 1961 8. Berge T, Linell F: Carcinoid tumours: Frequency in a defined population during a 12-year period. Acta Pathol Microbiol Scand [A] 84:322-330, 1976[Medline] 9. National Comprehensive Cancer Network: Clinical Practice Guidlines in Oncology: Neuroendocrine Tumors (ed v.1.2008). Fort Washington, PA, National Comprehensive Cancer Network, 2008, pp 1-32
Related Correspondence
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Copyright © 2008 by the American Society of Clinical Oncology, Online ISSN: 1527-7755. Print ISSN: 0732-183X
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2 cm.
