NCCN Expands Resources for Treating Rare Cancer Types

The National Comprehensive Cancer Network publishes two new NCCN Guidelines for Malignant Peritoneal Mesothelioma and for Ampullary Adenocarcinoma,to standardize treatment and improve outcomes for these rare tumor types; alsoreaches milestone expanding library of NCCN Chemotherapy Order Templates.

PLYMOUTH MEETING,Pa.,March 9,2022 --The National Comprehensive Cancer Network® (NCCN®)—an alliance of leading cancer centers—today announced the publication of new NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) for Ampullary Adenocarcinoma. This evidence- and expert consensus-based resource follows the recent publication of new NCCN Guidelines® for Malignant Peritoneal Mesothelioma,bringing the total number of clinical guidelines to 83.

NCCN announces new NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) for Ampullary Adenocarcinoma and Malignant Peritoneal Mesothelioma,bringing the total number of clinical guidelines to 83.

"We know there's a real need to share evidence-based expert recommendations for some of these rarer tumor types,which oncologists see infrequently and may not have the opportunity to keep as up to date with," said NCCN Chief Medical Officer Wui-Jin Koh,MD. "NCCN Guidelines were downloaded more than 13 million times overall in 2021. Guidelines for the most common cancers,including breast,lung,colon,and prostate tend to be referenced the most,but we do hear from clinicians who would like more guidance to help patients with less common cancers achieve the best possible outcomes."

NCCN Guidelines are the recognized standard for clinical recommendations and policy in cancer management and the most thorough and frequently-updated clinical practice guidelines available in any area of medicine. They are kept up-to-date by more than 1,700 subject matter experts from across the 31 NCCN Member Institutions,who contributed an estimated 40,000 hours across 60 different interdisciplinary panels over the last year. NCCN Guidelines are available free-of-charge for non-commercial use at NCCN.org or via the Virtual Library of NCCN Guidelines® App.

Early detection and prompt treatment can make a big difference in improving outcomes for ampullary tumors,which occur around a small opening at the junction of the duodenum,bile duct,and pancreatic duct. Ampullary adenocarcinoma accounts for less than one percent of all gastrointestinal malignancies,but tends to have a higher cure rate than other biliary tract and pancreatic cancers that may occur in the same general area.1-5

Malignant peritoneal mesothelioma (MPeM) is a rare,aggressive cancer that occurs in the lining of the abdomen (peritoneum) in about 600 patients every year in the United States. The new guidelines include an extensive section on the specific pathology tests that can be used to accurately identify MPeM,since it is challenging to diagnose due to its rarity and the fact that symptoms mimic other diseases like ovarian cancer. There is currently no recognized staging system for MPeM to assist with prognosis and treatment.6-8

"It can be hard for people with rare diseases to get the attention they deserve,but at NCCN we are doing all that we can to support people with any type of cancer,along with their loved ones and health care providers," said Dr. Koh. "The NCCN Guidelines currently cover 97 percent of cancer cases in the U.S.,and we'll keep adding more guidelines."

In addition to the growing library of clinical guidelines,NCCN recently published new and updated rare disease resource to empower patients and caregivers. Newly-posted NCCN Guidelines for Patients®: Systemic Mastocytosis (a rare mast cell disorder) and updated NCCN Guidelines for Patients: Small Cell Lung Cancer are available as a free download at NCCN.org/patientguidelines or the NCCN Patient Guides for Cancer App,or printed for a nominal fee via Amazon.com.

Another element in NCCN's efforts to improve patient care and safety for both rare and common cancers are the NCCN Chemotherapy Order Templates (NCCN Templates®),which recently surpassed 2,000 regimens. These resources provide user-friendly information on chemotherapy,immunotherapy,supportive care agents,monitoring parameters,and safety instructions,based on recommendations in the NCCN Guidelines. They help reduce medication errors and anticipate and manage potential adverse events,while standardizing patient care. Learn more at NCCN.org/ templates.

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About the National Comprehensive Cancer Network

The National Comprehensive Cancer Network® (NCCN®) is a not-for-profit alliance of leading cancer centers devoted to patient care,research,and education. NCCN is dedicated to improving and facilitating quality,effective,equitable,and accessible cancer care so all patients can live better lives. The NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) provide transparent,evidence-based,expert consensus recommendations for cancer treatment,prevention,and supportive services; they are the recognized standard for clinical direction and policy in cancer management and the most thorough and frequently-updated clinical practice guidelines available in any area of medicine. The NCCN Guidelines for Patients® provide expert cancer treatment information to inform and empower patients and caregivers,through support from the NCCN Foundation®. NCCN also advances continuing education,global initiatives,policy,and research collaboration and publication in oncology. Visit NCCN.org for more information and follow NCCN on Facebook @NCCNorg,Instagram @NCCNorg,and Twitter @NCCN.

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1Ahn DH,Bekaii-Saab T. Ampullary cancer: an overview. Am Soc Clin Oncol Educ Book 2014:112-115. Available at: https://www.ncbi.nlm.nih.gov/pubmed/24857067.

2 Jemal A,Siegel R,Ward E,et al. Cancer statistics,2008. CA Cancer J Clin 2008;58:71-96. Available at: https://www.ncbi.nlm.nih.gov/pubmed/18287387.

3 Westgaard A,Tafjord S,Farstad IN,et al. Pancreatobiliary versus intestinal histologic type of differentiation is an independent prognostic factor in resected periampullary adenocarcinoma. BMC Cancer 2008;8:170. Available at: https://www.ncbi.nlm.nih.gov/pubmed/18547417.

4Lemke J,Schafer D,Sander S,et al. Survival and prognostic factors in pancreatic and ampullary cancer. Anticancer Res 2014;34:3011-3020. Available at: https://www.ncbi.nlm.nih.gov/pubmed/24922667.

5Balachandran P,Sikora SS,Kapoor S,et al. Long-term survival and recurrence patterns in ampullary cancer. Pancreas 2006;32:390-395. Available at: https://www.ncbi.nlm.nih.gov/pubmed/16670621.

6Chicago Consensus Working Group. The Chicago Consensus on peritoneal surface malignancies: Management of peritoneal mesothelioma. Cancer. 2020;126(11):2547-2552.

7Howlader N,Noone AM,Krapcho M,et al (eds). SEER Cancer Statistics Review,1975-2018,National Cancer Institute. Bethesda,MD,https://seer.cancer.gov/csr/1975_2018/,based on November 2020 SEER data submission,posted to the SEER web site,April 2021.

8Ettinger DS,Wood DE,Stevenson J,et al. NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) for Malignant Peritoneal Mesothelioma (Version 1.2022). © 2021 National Comprehensive Cancer Network,Inc. To view the most recent and complete version of the NCCN Guidelines®,go online to NCCN.org.

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