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Targeted Therapy in a Young Adult With a Novel Epithelioid Tumor Driven by a PRRC2B-ALK Fusion

Ajay Gupta, Huifei Liu, Kathleen M. Schieffer, Selene C. Koo, Catherine E. Cottrell, Elaine R. Mardis, Ryan D. Roberts, and Nicholas D. Yeager

This case report describes an 18-year-old woman with an unusual epithelioid tumor of the omentum with a novel PRRC2B-ALK fusion. Although the atypical pathologic features raised significant diagnostic challenges, expression of CD30 on tumor cells and detection of an ALK rearrangement provided critical information for selecting targeted therapy in a patient not suitable for surgical resection. Despite an initially promising therapeutic response, the patient died. The efficacy of treatment was confirmed by the lack of viable tumor cells at autopsy. This case highlights the role of timely targeted therapy in patients with rare tumors and novel actionable molecular targets.

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Adolescent and Young Adult Oncology, Version 2.2018, NCCN Clinical Practice Guidelines in Oncology

Peter F. Coccia, Alberto S. Pappo, Lynda Beaupin, Virginia F. Borges, Scott C. Borinstein, Rashmi Chugh, Shira Dinner, Jeanelle Folbrecht, A. Lindsay Frazier, Robert Goldsby, Alexandra Gubin, Robert Hayashi, Mary S. Huang, Michael P. Link, John A. Livingston, Yousif Matloub, Frederick Millard, Kevin C. Oeffinger, Diane Puccetti, Damon Reed, Steven Robinson, Abby R. Rosenberg, Tara Sanft, Holly L. Spraker-Perlman, Margaret von Mehren, Daniel S. Wechsler, Kimberly F. Whelan, Nicholas Yeager, Lisa A. Gurski, and Dorothy A. Shead

This selection from the NCCN Guidelines for Adolescent and Young Adult (AYA) Oncology focuses on treatment and management considerations for AYA patients with cancer. Compared with older adults with cancer, AYA patients have unique needs regarding treatment, fertility counseling, psychosocial and behavioral issues, and supportive care services. The complete version of the NCCN Guidelines for AYA Oncology addresses additional aspects of caring for AYA patients, including risk factors, screening, diagnosis, and survivorship.

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Adolescent and Young Adult (AYA) Oncology, Version 2.2024, NCCN Clinical Practice Guidelines in Oncology

Smita Bhatia, Alberto S. Pappo, Melissa Acquazzino, Wendy A. Allen-Rhoades, Marie Barnett, Scott C. Borinstein, Robert Casey, Sun Choo, Rashmi Chugh, Shira Dinner, Ralph Ermoian, Douglas Fair, Noah Federman, Jeanelle Folbrecht, Shipra Gandhi, Julie Germann, Robert Goldsby, Robert Hayashi, Alex Y. Huang, Mary S. Huang, Linda A. Jacobs, Cathy Lee-Miller, Michael P. Link, John A. Livingston, Maryam Lustberg, Marcio Malogolowkin, Kevin C. Oeffinger, Christine A. Pratilas, Damon Reed, Jodi Skiles, Margaret von Mehren, Nicholas Yeager, Sarah Montgomery, and Lisa Hang

This selection from the NCCN Guidelines for Adolescent and Young Adult (AYA) Oncology focuses on considerations for the comprehensive care of AYA patients with cancer. Compared with older adults with cancer, AYA patients have unique needs regarding treatment, fertility counseling, psychosocial and behavioral issues, and supportive care services. The complete version of the NCCN Guidelines for Adolescent and Young Adult (AYA) Oncology addresses additional aspects of caring for AYA patients, including risk factors, screening, diagnosis, and survivorship.