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Karam Khaddour, Michael R. Chicoine, Jiayi Huang, Sonika Dahiya, and George Ansstas

Craniopharyngiomas are rare tumors that arise in the suprasellar region of the brain and are known for their aggressive nature despite their WHO grade I. This is due to the complex neuroanatomy of the sellar/suprasellar region and their proximity to the optic nerve apparatus, hypothalamic–pituitary tract, and other critical neuroanatomical structures. Definitive treatment is based on a multidisciplinary approach and often involves a combination of surgical, radiation, and medical therapy. However, there is high morbidity associated with surgery and RT due to the complex neuroanatomy of this region. Recently, BRAFV600E somatic mutation has been identified in most papillary craniopharyngiomas. This discovery has led to the novel use of RAF pathway inhibitors to treat these tumors. We report the successful use of dabrafenib (BRAF inhibitor) and trametinib (mitogen-activated protein kinase kinase inhibitor) in the neoadjuvant setting followed by definitive stereotactic radiosurgery. We propose an algorithm based on available literature on the integration of targeted therapy in the management of papillary craniopharyngiomas. Our observations, together with prior case reports, advocate the incorporation of targeted therapy for unresectable craniopharyngiomas and reinforce that treatment with dual-targeted therapy is safe and effective.

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Richard T. Hoppe, Ranjana H. Advani, Weiyun Z. Ai, Richard F. Ambinder, Patricia Aoun, Celeste M. Bello, Cecil M. Benitez, Karl Bernat, Philip J. Bierman, Kristie A. Blum, Robert Chen, Bouthaina Dabaja, 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, David G. Maloney, Peter M. Mauch, Monika Metzger, Joseph O. Moore, David Morgan, Craig H. Moskowitz, Carolyn Mulroney, Matthew Poppe, Rachel Rabinovitch, Stuart Seropian, Mitchell Smith, Jane N. Winter, Joachim Yahalom, Jennifer Burns, Ndiya Ogba, and Hema Sundar

This portion of the NCCN Guidelines for Hodgkin lymphoma (HL) focuses on the management of classical HL. Current management of classical HL involves initial treatment with chemotherapy or combined modality therapy followed by restaging with PET/CT to assess treatment response using the Deauville criteria (5-point scale). The introduction of less toxic and more effective regimens has significantly advanced HL cure rates. However, long-term follow-up after completion of treatment is essential to determine potential long-term effects.

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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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Richard T. Hoppe, Ranjana H. Advani, Weiyun Z. Ai, Richard F. Ambinder, Patricia Aoun, Celeste M. Bello, Cecil M. Benitez, Philip J. Bierman, Kristie A. Blum, Robert Chen, Bouthaina Dabaja, Andres Forero, Leo I. Gordon, Francisco J. Hernandez-Ilizaliturri, Ephraim P. Hochberg, Jiayi Huang, Patrick B. Johnston, Nadia Khan, David G. Maloney, Peter M. Mauch, Monika Metzger, Joseph O. Moore, David Morgan, Craig H. Moskowitz, Carolyn Mulroney, Matthew Poppe, Rachel Rabinovitch, Stuart Seropian, Christina Tsien, Jane N. Winter, Joachim Yahalom, Jennifer L. Burns, and Hema Sundar

Hodgkin lymphoma (HL) is an uncommon malignancy involving lymph nodes and the lymphatic system. Classical Hodgkin lymphoma (CHL) and nodular lymphocyte-predominant Hodgkin lymphoma are the 2 main types of HL. CHL accounts for most HL diagnosed in the Western countries. Chemotherapy or combined modality therapy, followed by restaging with PET/CT to assess treatment response using the Deauville criteria (5-point scale), is the standard initial treatment for patients with newly diagnosed CHL. Brentuximab vedotin, a CD30-directed antibody-drug conjugate, has produced encouraging results in the treatment of relapsed or refractory disease. The potential long-term effects of treatment remain an important consideration, and long-term follow-up is essential after completion of treatment.

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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.