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Boyu Hu, Jay L. Patel, Randa Tao, Richard B. Cannon, Marcus Monroe, and Gaurav Goyal

Survival outcomes of patients with histiocytic neoplasms are poor, with no standard-of-care treatments available for these malignancies. Recent characterization of the genomic landscape of various histiocytic neoplasms have shown a predominance of activating driver mutations within the MAPK/ERK pathway (ie, BRAF, MEK, KRAS, MAPK, and NRAS). Subsequently, successful treatment of these malignancies with BRAF and MEK inhibitors has been reported. This report presents the first patient with histiocytic sarcoma harboring a somatic KRAS Q61H mutation who was subsequently treated to a near complete response with the MEK inhibitor trametinib. Due to patient preference, lack of standard of care treatments, and associated morbidity from head and neck dissection, initial disease reduction provided by trametinib therapy allowed for a less morbid resection. This case report highlights the utility of up-front next-generation sequencing and the efficacy of MEK inhibition in patients with histiocytic sarcoma harboring activating KRAS mutations.

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Richard T. Hoppe, Ranjana H. Advani, Weiyun Z. Ai, Richard F. Ambinder, Philippe Armand, Celeste M. Bello, Cecil M. Benitez, Philip J. Bierman, Kirsten M. Boughan, Bouthaina Dabaja, Leo I. Gordon, Francisco J. Hernandez-Ilizaliturri, Alex F. Herrera, Ephraim P. Hochberg, Jiayi Huang, Patrick B. Johnston, Mark S. Kaminski, Vaishalee P. Kenkre, Nadia Khan, Ryan C. Lynch, Kami Maddocks, Jonathan McConathy, Matthew McKinney, Monika Metzger, David Morgan, Carolyn Mulroney, Rachel Rabinovitch, Karen C. Rosenspire, Stuart Seropian, Randa Tao, Jane N. Winter, Joachim Yahalom, Jennifer L. Burns, and Ndiya Ogba

The NCCN Clinical Practice Guidelines in Oncology for Hodgkin Lymphoma (HL) provide recommendations for the management of adult patients with HL. The NCCN panel meets at least annually to review comments from reviewers within their institutions, examine relevant data, and reevaluate and update their recommendations. Current management of classic HL involves initial treatment with chemotherapy alone or combined modality therapy followed by restaging with PET/CT to assess treatment response. Overall, the introduction of less toxic and more effective regimens has significantly advanced HL cure rates. This portion of the NCCN Guidelines focuses on the management of classic HL.

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NCCN Guidelines® Insights: Hodgkin Lymphoma, Version 2.2022

Featured Updates to the NCCN Guidelines

Richard T. Hoppe, Ranjana H. Advani, Weiyun Z. Ai, Richard F. Ambinder, Philippe Armand, Celeste M. Bello, Cecil M. Benitez, Weina Chen, Bouthaina Dabaja, Megan E. Daly, Leo I. Gordon, Neil Hansen, Alex F. Herrera, Ephraim P. Hochberg, Patrick B. Johnston, Mark S. Kaminski, Christopher R. Kelsey, Vaishalee P. Kenkre, Nadia Khan, Ryan C. Lynch, Kami Maddocks, Jonathan McConathy, Monika Metzger, David Morgan, Carolyn Mulroney, Sheeja T. Pullarkat, Rachel Rabinovitch, Karen C. Rosenspire, Stuart Seropian, Randa Tao, Pallawi Torka, Jane N. Winter, Joachim Yahalom, Joanna C. Yang, Jennifer L. Burns, Mallory Campbell, and Hema Sundar

Hodgkin lymphoma (HL) is an uncommon malignancy of B-cell origin. Classical HL (cHL) and nodular lymphocyte–predominant HL are the 2 main types of HL. The cure rates for HL have increased so markedly with the advent of modern treatment options that overriding treatment considerations often relate to long-term toxicity. These NCCN Guidelines Insights discuss the recent updates to the NCCN Guidelines for HL focusing on (1) radiation therapy dose constraints in the management of patients with HL, and (2) the management of advanced-stage and relapsed or refractory cHL.

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Richard T. Hoppe, Ranjana H. Advani, Weiyun Z. Ai, Richard F. Ambinder, Patricia Aoun, Philippe Armand, Celeste M. Bello, Cecil M. Benitez, Philip J. Bierman, Robert Chen, Bouthaina Dabaja, Robert Dean, Andres Forero, Leo I. Gordon, Francisco J. Hernandez-Ilizaliturri, Ephraim P. Hochberg, Jiayi Huang, Patrick B. Johnston, Mark S. Kaminski, Vaishalee P. Kenkre, Nadia Khan, Kami Maddocks, David G. Maloney, Monika Metzger, Joseph O. Moore, David Morgan, Craig H. Moskowitz, Carolyn Mulroney, Rachel Rabinovitch, Stuart Seropian, Randa Tao, Jane N. Winter, Joachim Yahalom, Jennifer L. Burns, and Ndiya Ogba

The NCCN Clinical Practice Guidelines in Oncology for Hodgkin Lymphoma (HL) provide recommendations for the management of adult patients with HL. The NCCN Guidelines Panel meets at least annually to review comments from reviewers within the NCCN Member Institutions, examine relevant data, and reevaluate and update the recommendations. These NCCN Guidelines Insights summarize recent updates centered on treatment considerations for relapsed/refractory classic HL.