Clinicopathologic Features, Management, and Outcomes Of Pediatric CRTC1::TRIM11 and MED15::ATF1 Tumors With Spitzoid Morphology: A Case Series

作者：Michael R. Sargen, Madeline H. Fitzhugh, Jennifer S. Ko, Allison Close, Sara C. Horton, Faizan Malik, Larissa V. Furtado, Armita Bahrami, Brittani Seynnaeve, Alberto S. Pappo, Vincent Liu, Kmc Cheung, Svetlana Pack, Liqiang Xi, Mark Raffeld, Ina Lee, Jeffrey Gagan, Kenneth Aldape, Frederic G. Barr, Stephen M. Hewitt, Christina K. Ferrone, Anna Hayden, Jessica N. Hatton, Alisa M. Goldstein, Ruth M. Pfeiffer, Brigitte C. Widemann, Mary Frances Wedekind, Andrea M. Gross, Rosandra N. Kaplan, Marielle E. Yohe, Kristen G. Berrebi · 发表于：Pediatric Blood & Cancer · 年份：2025 · DOI：10.1002/pbc.31814 · 被引用次数：8 · 研究领域：Immunotherapy and Immune Responses、interferon and immune responses、Viral-associated cancers and disorders

Limited information is available on the clinicopathologic features and management of pediatric CRTC1::TRIM11 (CTRTs) and MED15::ATF1-rearranged tumors (MARTs). In this series of pediatric CTRTs (n = 5) and MARTs (n = 2), all tumors exhibited spitzoid morphology and expressed melanocytic markers. Metastatic progression occurred in one CTRT and two MARTs: all three received immunotherapy, resulting in disease control in two patients and one death due to progressive disease. A fourth individual had lymph node micrometastasis that remained stable without progression. This case series highlights the potentially aggressive behavior of these tumors and suggests that immunotherapy may be an effective treatment option for advanced disease.