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Medhavi Gupta, Christopher Sherrow, Maghan E. Krone, Edik M. Blais, Michael J. Pishvaian, Emanuel F. Petricoin III, Lynn M. Matrisian, Patricia DeArbeloa, Gary Gregory, Alyson Brown, Olivia Zalewski, Gillian Prinzing, Charles Roche, Kazunori Kanehira, Sarbajit Mukherjee, Renuka Iyer, and Christos Fountzilas

Pancreatic acinar cell carcinoma (PACC) is a rare pancreatic exocrine malignancy. Compared with the more common pancreatic ductal adenocarcinoma (PDAC), PACC is more common in younger White men, has earlier stages and a lower mean age (56 vs 70 years) at the time of presentation, and has a better prognosis. In addition to differences in demographic, histologic, and clinical characteristics, PACC has a genomic profile distinct from PDAC, with only rare mutations in TP53, KRAS, and p16 that are commonly found in PDAC. This case report presents a man aged 81 years who presented with a pancreatic body mass with peripancreatic lymph node enlargement. Biopsy of the mass showed acinar cell carcinoma. The patient underwent upfront surgical resection, followed by one cycle of adjuvant gemcitabine, with stoppage of therapy due to poor tolerance. Lower-dose gemcitabine was reintroduced after disease progression 6 months later. Nab-paclitaxel was added to gemcitabine after 6 cycles because of a continued increase in the size of peripancreatic lymph nodes. Combination chemotherapy was stopped after 4 cycles because of further disease progression with new liver metastasis. Molecular testing showed the presence of an SEL1L-NTRK1 fusion. Targeted therapy was started with the oral neurotrophic tropomyosin receptor kinase (NTRK) inhibitor larotrectinib at a dosage of 100 mg twice daily. At the time of writing, the patient has been on therapy for 13 months with an exceptional radiographic response and has not experienced any grade 3 adverse effects. To our knowledge, this is the first clinical report of an NTRK gene fusion in a patient with PACC. This case study highlights the significance of tumor molecular profiling in patients with pancreatic tumors, especially rare histologies.

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Margaret A. Tempero, Mokenge P. Malafa, E. Gabriela Chiorean, Brian Czito, Courtney Scaife, Amol K. Narang, Christos Fountzilas, Brian M. Wolpin, Mahmoud Al-Hawary, Horacio Asbun, Stephen W. Behrman, Al B. Benson III, Ellen Binder, Dana B. Cardin, Charles Cha, Vincent Chung, Mary Dillhoff, Efrat Dotan, Cristina R. Ferrone, George Fisher, Jeffrey Hardacre, William G. Hawkins, Andrew H. Ko, Noelle LoConte, Andrew M. Lowy, Cassadie Moravek, Eric K. Nakakura, Eileen M. O’Reilly, Jorge Obando, Sushanth Reddy, Sarah Thayer, Robert A. Wolff, Jennifer L. Burns, and Griselda Zuccarino-Catania

The NCCN Guidelines for Pancreatic Adenocarcinoma discuss the diagnosis and management of adenocarcinomas of the exocrine pancreas and are intended to assist with clinical decision-making. These NCCN Guidelines Insights discuss important updates to the 2019 version of the guidelines, focusing on postoperative adjuvant treatment of patients with pancreatic cancers.

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Margaret A. Tempero, Mokenge P. Malafa, Mahmoud Al-Hawary, Stephen W. Behrman, Al B. Benson III, Dana B. Cardin, E. Gabriela Chiorean, Vincent Chung, Brian Czito, Marco Del Chiaro, Mary Dillhoff, Timothy R. Donahue, Efrat Dotan, Cristina R. Ferrone, Christos Fountzilas, Jeffrey Hardacre, William G. Hawkins, Kelsey Klute, Andrew H. Ko, John W. Kunstman, Noelle LoConte, Andrew M. Lowy, Cassadie Moravek, Eric K. Nakakura, Amol K. Narang, Jorge Obando, Patricio M. Polanco, Sushanth Reddy, Marsha Reyngold, Courtney Scaife, Jeanne Shen, Charles Vollmer Jr., Robert A. Wolff, Brian M. Wolpin, Beth Lynn, and Giby V. George

Pancreatic cancer is the fourth leading cause of cancer-related death among men and women in the United States. A major challenge in treatment remains patients’ advanced disease at diagnosis. The NCCN Guidelines for Pancreatic Adenocarcinoma provides recommendations for the diagnosis, evaluation, treatment, and follow-up for patients with pancreatic cancer. Although survival rates remain relatively unchanged, newer modalities of treatment, including targeted therapies, provide hope for improving patient outcomes. Sections of the manuscript have been updated to be concordant with the most recent update to the guidelines. This manuscript focuses on the available systemic therapy approaches, specifically the treatment options for locally advanced and metastatic disease.