title: Systemic therapy for advanced appendiceal adenocarcinoma: an analysis from the NCCN Oncology Outcomes Database for colorectal cancer.
abstract: Appendiceal malignancies are rare and represent 1% of intestinal tumors in the United States. The role and efficacy of modern systemic therapy in advanced appendiceal adenocarcinoma has not been established. This study analyzed patients with recurrent or metastatic appendiceal adenocarcinoma in the database for Colorectal Cancer (CRC; 2005-2012). This database tracks longitudinal care for patients treated at 8 specialty centers across the Unites States. Study objectives were to describe and evaluate the efficacy of systemic therapy and investigate relationships with clinicopathologic features. Cox regression analysis was performed to identify predictors of progression-free survival (PFS) and overall survival (OS). Of 248 patients with advanced appendiceal carcinoma, 112 (45%) received systemic therapy for measurable disease and are the focus of this report. The most common chemotherapy regimens included FOLFOX with or without bevacizumab (n=39 and n=37, respectively), FOLFIRI (n=15), and single-agent fluoro-pyrimidine (n=10). Among 99 patients evaluable for best response, 39 experienced a response (response rate [RR], 39%) and 36 (36%) had stable disease. The median PFS was 1.2 years (95% CI, 1.0-1.8) and median OS was 2.1 years (95% CI, 1.6-2.3). Patients with non-mucinous histology or high-grade tumors and those who underwent nondebulking surgery had worse PFS and OS. Treatment of advanced appendiceal adenocarcinoma at NCCN Member Institutions commonly incorporates agents used for CRC. RR, PFS, and OS are comparable to those achieved in the treatment of metastatic CRC. Poor prognostic factors include nonmucinous histology or high-grade tumors and history of nondebulking surgery.
authors.affiliation: From Fox Chase Cancer Center, Philadelphia, Pennsylvania; City of Hope Comprehensive Cancer Center, Duarte, California; Dana-Farber Cancer Institute, Boston, Massachusetts; The Ohio State University Comprehensive Cancer Center - James Cancer Hospital and Solove Research Institute, Columbus, Ohio; Robert H. Lurie Comprehensive Cancer Center of Northwestern University, Chicago, Illinois; The University of Texas MD Anderson Cancer Center, Houston, Texas; Memorial Sloan Kettering Cancer Center, New York, New York; and Roswell Park Cancer Institute, Buffalo, New York.
authors.forename: Mohamedtaki A
authors.initials: MA
authors.lastname: Tejani
authors.affiliation: From Fox Chase Cancer Center, Philadelphia, Pennsylvania; City of Hope Comprehensive Cancer Center, Duarte, California; Dana-Farber Cancer Institute, Boston, Massachusetts; The Ohio State University Comprehensive Cancer Center - James Cancer Hospital and Solove Research Institute, Columbus, Ohio; Robert H. Lurie Comprehensive Cancer Center of Northwestern University, Chicago, Illinois; The University of Texas MD Anderson Cancer Center, Houston, Texas; Memorial Sloan Kettering Cancer Center, New York, New York; and Roswell Park Cancer Institute, Buffalo, New York.
authors.forename: Anna
authors.initials: A
authors.lastname: ter Veer
authors.affiliation: From Fox Chase Cancer Center, Philadelphia, Pennsylvania; City of Hope Comprehensive Cancer Center, Duarte, California; Dana-Farber Cancer Institute, Boston, Massachusetts; The Ohio State University Comprehensive Cancer Center - James Cancer Hospital and Solove Research Institute, Columbus, Ohio; Robert H. Lurie Comprehensive Cancer Center of Northwestern University, Chicago, Illinois; The University of Texas MD Anderson Cancer Center, Houston, Texas; Memorial Sloan Kettering Cancer Center, New York, New York; and Roswell Park Cancer Institute, Buffalo, New York.
authors.forename: Dana
authors.initials: D
authors.lastname: Milne
authors.affiliation: From Fox Chase Cancer Center, Philadelphia, Pennsylvania; City of Hope Comprehensive Cancer Center, Duarte, California; Dana-Farber Cancer Institute, Boston, Massachusetts; The Ohio State University Comprehensive Cancer Center - James Cancer Hospital and Solove Research Institute, Columbus, Ohio; Robert H. Lurie Comprehensive Cancer Center of Northwestern University, Chicago, Illinois; The University of Texas MD Anderson Cancer Center, Houston, Texas; Memorial Sloan Kettering Cancer Center, New York, New York; and Roswell Park Cancer Institute, Buffalo, New York.
authors.forename: Rebecca
authors.initials: R
authors.lastname: Ottesen
authors.affiliation: From Fox Chase Cancer Center, Philadelphia, Pennsylvania; City of Hope Comprehensive Cancer Center, Duarte, California; Dana-Farber Cancer Institute, Boston, Massachusetts; The Ohio State University Comprehensive Cancer Center - James Cancer Hospital and Solove Research Institute, Columbus, Ohio; Robert H. Lurie Comprehensive Cancer Center of Northwestern University, Chicago, Illinois; The University of Texas MD Anderson Cancer Center, Houston, Texas; Memorial Sloan Kettering Cancer Center, New York, New York; and Roswell Park Cancer Institute, Buffalo, New York.
authors.forename: Tanios
authors.initials: T
authors.lastname: Bekaii-Saab
authors.affiliation: From Fox Chase Cancer Center, Philadelphia, Pennsylvania; City of Hope Comprehensive Cancer Center, Duarte, California; Dana-Farber Cancer Institute, Boston, Massachusetts; The Ohio State University Comprehensive Cancer Center - James Cancer Hospital and Solove Research Institute, Columbus, Ohio; Robert H. Lurie Comprehensive Cancer Center of Northwestern University, Chicago, Illinois; The University of Texas MD Anderson Cancer Center, Houston, Texas; Memorial Sloan Kettering Cancer Center, New York, New York; and Roswell Park Cancer Institute, Buffalo, New York.
authors.forename: Al B
authors.initials: AB
authors.lastname: Benson
authors.affiliation: From Fox Chase Cancer Center, Philadelphia, Pennsylvania; City of Hope Comprehensive Cancer Center, Duarte, California; Dana-Farber Cancer Institute, Boston, Massachusetts; The Ohio State University Comprehensive Cancer Center - James Cancer Hospital and Solove Research Institute, Columbus, Ohio; Robert H. Lurie Comprehensive Cancer Center of Northwestern University, Chicago, Illinois; The University of Texas MD Anderson Cancer Center, Houston, Texas; Memorial Sloan Kettering Cancer Center, New York, New York; and Roswell Park Cancer Institute, Buffalo, New York.
authors.forename: Deborah
authors.initials: D
authors.lastname: Schrag
authors.affiliation: From Fox Chase Cancer Center, Philadelphia, Pennsylvania; City of Hope Comprehensive Cancer Center, Duarte, California; Dana-Farber Cancer Institute, Boston, Massachusetts; The Ohio State University Comprehensive Cancer Center - James Cancer Hospital and Solove Research Institute, Columbus, Ohio; Robert H. Lurie Comprehensive Cancer Center of Northwestern University, Chicago, Illinois; The University of Texas MD Anderson Cancer Center, Houston, Texas; Memorial Sloan Kettering Cancer Center, New York, New York; and Roswell Park Cancer Institute, Buffalo, New York.
authors.forename: Stephen
authors.initials: S
authors.lastname: Shibata
authors.affiliation: From Fox Chase Cancer Center, Philadelphia, Pennsylvania; City of Hope Comprehensive Cancer Center, Duarte, California; Dana-Farber Cancer Institute, Boston, Massachusetts; The Ohio State University Comprehensive Cancer Center - James Cancer Hospital and Solove Research Institute, Columbus, Ohio; Robert H. Lurie Comprehensive Cancer Center of Northwestern University, Chicago, Illinois; The University of Texas MD Anderson Cancer Center, Houston, Texas; Memorial Sloan Kettering Cancer Center, New York, New York; and Roswell Park Cancer Institute, Buffalo, New York.
authors.forename: John
authors.initials: J
authors.lastname: Skibber
authors.affiliation: From Fox Chase Cancer Center, Philadelphia, Pennsylvania; City of Hope Comprehensive Cancer Center, Duarte, California; Dana-Farber Cancer Institute, Boston, Massachusetts; The Ohio State University Comprehensive Cancer Center - James Cancer Hospital and Solove Research Institute, Columbus, Ohio; Robert H. Lurie Comprehensive Cancer Center of Northwestern University, Chicago, Illinois; The University of Texas MD Anderson Cancer Center, Houston, Texas; Memorial Sloan Kettering Cancer Center, New York, New York; and Roswell Park Cancer Institute, Buffalo, New York.
authors.forename: Martin
authors.initials: M
authors.lastname: Weiser
authors.affiliation: From Fox Chase Cancer Center, Philadelphia, Pennsylvania; City of Hope Comprehensive Cancer Center, Duarte, California; Dana-Farber Cancer Institute, Boston, Massachusetts; The Ohio State University Comprehensive Cancer Center - James Cancer Hospital and Solove Research Institute, Columbus, Ohio; Robert H. Lurie Comprehensive Cancer Center of Northwestern University, Chicago, Illinois; The University of Texas MD Anderson Cancer Center, Houston, Texas; Memorial Sloan Kettering Cancer Center, New York, New York; and Roswell Park Cancer Institute, Buffalo, New York.
authors.forename: Neal
authors.initials: N
authors.lastname: Wilkinson
authors.affiliation: From Fox Chase Cancer Center, Philadelphia, Pennsylvania; City of Hope Comprehensive Cancer Center, Duarte, California; Dana-Farber Cancer Institute, Boston, Massachusetts; The Ohio State University Comprehensive Cancer Center - James Cancer Hospital and Solove Research Institute, Columbus, Ohio; Robert H. Lurie Comprehensive Cancer Center of Northwestern University, Chicago, Illinois; The University of Texas MD Anderson Cancer Center, Houston, Texas; Memorial Sloan Kettering Cancer Center, New York, New York; and Roswell Park Cancer Institute, Buffalo, New York.
authors.forename: Steven J
authors.initials: SJ
authors.lastname: Cohen
mesh_terms: D000230:Adenocarcinoma / Q000188:drug therapy* / Q000473:pathology; D000328:Adult; D000368:Aged; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000008:administration & dosage*; D001063:Appendiceal Neoplasms / Q000188:drug therapy* / Q000473:pathology; D015179:Colorectal Neoplasms / Q000188:drug therapy / Q000473:pathology; D018572:Disease-Free Survival; D005260:Female; D005472:Fluorouracil / Q000008:administration & dosage; D006801:Humans; D053208:Kaplan-Meier Estimate; D002955:Leucovorin / Q000008:administration & dosage; D008297:Male; D008875:Middle Aged; D009944:Organoplatinum Compounds / Q000008:administration & dosage; D016896:Treatment Outcome; D014481:United States
chemical_list: D009944:Organoplatinum Compounds; D002955:Leucovorin; D005472:Fluorouracil