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Maha Alkhuziem, Adam M. Burgoyne, Paul T. Fanta, Chih-Min Tang and Jason K. Sicklick

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Lindsey M. Charo, Adam M. Burgoyne, Paul T. Fanta, Hitendra Patel, Juliann Chmielecki, Jason K. Sicklick and Michael T. McHale

Gastrointestinal stromal tumors (GISTs) are rare in pregnancy, with only 11 reported cases. Adjuvant imatinib therapy, which targets the most common driver mutations in GIST (KIT and PDGFRA), is recommended for patients with high-risk GIST, but it has known teratogenicity in the first trimester. A 34-year-old G3P2 woman underwent exploratory laparotomy at 16 weeks' gestation for a presumed adnexal mass. Surgical findings included normal adnexa and a 14-cm solid small bowel mass. The mass was resected en bloc with a segment of jejunum followed by a primary anastomosis. Histopathology and genomic analyses demonstrated a GIST with high-risk features but lack of KIT/PDGFRA mutations and identified the presence of a previously unreported, pathogenic PRKAR1B-BRAF gene fusion. Given her tumor profile, adjuvant therapy with imatinib was not recommended. GIST is rare in pregnancy, but can masquerade as an adnexal mass in women of childbearing age. Because neoadjuvant/adjuvant imatinib has risks of teratogenicity, tumor molecular profiling is critical as we identified a previously unreported gene fusion of PRKAR1B with BRAF that is predicted to be imatinib-resistant. In this case, testing provided the rationale for not offering adjuvant imatinib to avoid unnecessary toxicity to the patient and fetus.

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Matthew H. Kulke, Manisha H. Shah, Al B. Benson III, Emily Bergsland, Jordan D. Berlin, Lawrence S. Blaszkowsky, Lyska Emerson, Paul F. Engstrom, Paul Fanta, Thomas Giordano, Whitney S. Goldner, Thorvardur R. Halfdanarson, Martin J. Heslin, Fouad Kandeel, Pamela L. Kunz, Boris W. Kuvshinoff II, Christopher Lieu, Jeffrey F. Moley, Gitonga Munene, Venu G. Pillarisetty, Leonard Saltz, Julie Ann Sosa, Jonathan R. Strosberg, Jean-Nicolas Vauthey, Christopher Wolfgang, James C. Yao, Jennifer Burns and Deborah Freedman-Cass

Neuroendocrine tumors (NETs) comprise a broad family of tumors that may or may not be associated with symptoms attributable to hormonal hypersecretion. The NCCN Clinical Practice Guidelines in Oncology for Neuroendocrine Tumors discuss the diagnosis and management of both sporadic and hereditary NETs. This selection from the guidelines focuses on sporadic NETs of the pancreas, gastrointestinal tract, lung, and thymus.

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Jaffer A. Ajani, Thomas A. D'Amico, Khaldoun Almhanna, David J. Bentrem, Joseph Chao, Prajnan Das, Crystal S. Denlinger, Paul Fanta, Farhood Farjah, Charles S. Fuchs, Hans Gerdes, Michael Gibson, Robert E. Glasgow, James A. Hayman, Steven Hochwald, Wayne L. Hofstetter, David H. Ilson, Dawn Jaroszewski, Kimberly L. Johung, Rajesh N. Keswani, Lawrence R. Kleinberg, W. Michael Korn, Stephen Leong, Catherine Linn, A. Craig Lockhart, Quan P. Ly, Mary F. Mulcahy, Mark B. Orringer, Kyle A. Perry, George A. Poultsides, Walter J. Scott, Vivian E. Strong, Mary Kay Washington, Benny Weksler, Christopher G. Willett, Cameron D. Wright, Debra Zelman, Nicole McMillian and Hema Sundar

Gastric cancer is the fifth most frequently diagnosed cancer and the third leading cause of death from cancer in the world. Several advances have been made in the staging procedures, imaging techniques, and treatment approaches. The NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines) for Gastric Cancer provide an evidence- and consensus-based treatment approach for the management of patients with gastric cancer. This manuscript discusses the recommendations outlined in the NCCN Guidelines for staging, assessment of HER2 overexpression, systemic therapy for locally advanced or metastatic disease, and best supportive care for the prevention and management of symptoms due to advanced disease.

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Jaffer A. Ajani, Thomas A. D’Amico, Khaldoun Almhanna, David J. Bentrem, Stephen Besh, Joseph Chao, Prajnan Das, Crystal Denlinger, Paul Fanta, Charles S. Fuchs, Hans Gerdes, Robert E. Glasgow, James A. Hayman, Steven Hochwald, Wayne L. Hofstetter, David H. Ilson, Dawn Jaroszewski, Kory Jasperson, Rajesh N. Keswani, Lawrence R. Kleinberg, W. Michael Korn, Stephen Leong, A. Craig Lockhart, Mary F. Mulcahy, Mark B. Orringer, James A. Posey, George A. Poultsides, Aaron R. Sasson, Walter J. Scott, Vivian E. Strong, Thomas K. Varghese Jr, Mary Kay Washington, Christopher G. Willett, Cameron D. Wright, Debra Zelman, Nicole McMillian and Hema Sundar

Esophageal cancer is the sixth most common cause of cancer deaths worldwide. Adenocarcinoma is more common in North America and Western European countries, originating mostly in the lower third of the esophagus, which often involves the esophagogastric junction (EGJ). Recent randomized trials have shown that the addition of preoperative chemoradiation or perioperative chemotherapy to surgery significantly improves survival in patients with resectable cancer. Targeted therapies with trastuzumab and ramucirumab have produced encouraging results in the treatment of advanced or metastatic EGJ adenocarcinomas. Multidisciplinary team management is essential for patients with esophageal and EGJ cancers. This portion of the NCCN Guidelines for Esophageal and EGJ Cancers discusses management of locally advanced adenocarcinoma of the esophagus and EGJ.

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Manisha H. Shah, Whitney S. Goldner, Thorvardur R. Halfdanarson, Emily Bergsland, Jordan D. Berlin, Daniel Halperin, Jennifer Chan, Matthew H. Kulke, Al B. Benson III, Lawrence S. Blaszkowsky, Jennifer Eads, Paul F. Engstrom, Paul Fanta, Thomas Giordano, Jin He, Martin J. Heslin, Gregory P. Kalemkerian, Fouad Kandeel, Sajid A. Khan, Wajih Zaheer Kidwai, Pamela L. Kunz, Boris W. Kuvshinoff II, Christopher Lieu, Venu G. Pillarisetty, Leonard Saltz, Julie Ann Sosa, Jonathan R. Strosberg, Craig A. Sussman, Nikolaos A. Trikalinos, Nataliya A. Uboha, Jonathan Whisenant, Terence Wong, James C. Yao, Jennifer L. Burns, Ndiya Ogba and Griselda Zuccarino-Catania

The NCCN Guidelines for Neuroendocrine and Adrenal Tumors provide recommendations for the management of adult patients with neuroendocrine tumors (NETs), adrenal gland tumors, pheochromocytomas, and paragangliomas. Management of NETs relies heavily on the site of the primary NET. These NCCN Guidelines Insights summarize the management options and the 2018 updates to the guidelines for locoregional advanced disease, and/or distant metastasis originating from gastrointestinal tract, bronchopulmonary, and thymus primary NETs.

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Jaffer A. Ajani, Thomas A. D’Amico, David J. Bentrem, Joseph Chao, Carlos Corvera, Prajnan Das, Crystal S. Denlinger, Peter C. Enzinger, Paul Fanta, Farhood Farjah, Hans Gerdes, Michael Gibson, Robert E. Glasgow, James A. Hayman, Steven Hochwald, Wayne L. Hofstetter, David H. Ilson, Dawn Jaroszewski, Kimberly L. Johung, Rajesh N. Keswani, Lawrence R. Kleinberg, Stephen Leong, Quan P. Ly, Kristina A. Matkowskyj, Michael McNamara, Mary F. Mulcahy, Ravi K. Paluri, Haeseong Park, Kyle A. Perry, Jose Pimiento, George A. Poultsides, Robert Roses, Vivian E. Strong, Georgia Wiesner, Christopher G. Willett, Cameron D. Wright, Nicole R. McMillian and Lenora A. Pluchino

Abstract

Esophageal cancer is the sixth leading cause of cancer-related deaths worldwide. Squamous cell carcinoma is the most common histology in Eastern Europe and Asia, and adenocarcinoma is most common in North America and Western Europe. Surgery is a major component of treatment of locally advanced resectable esophageal and esophagogastric junction (EGJ) cancer, and randomized trials have shown that the addition of preoperative chemoradiation or perioperative chemotherapy to surgery significantly improves survival. Targeted therapies including trastuzumab, ramucirumab, and pembrolizumab have produced encouraging results in the treatment of patients with advanced or metastatic disease. Multidisciplinary team management is essential for all patients with esophageal and EGJ cancers. This selection from the NCCN Guidelines for Esophageal and Esophagogastric Junction Cancers focuses on recommendations for the management of locally advanced and metastatic adenocarcinoma of the esophagus and EGJ.