具有Spitz样形态的儿童CRTC1::TRIM11和MED15::ATF1肿瘤的临床病理特征、管理及预后：病例系列
Clinicopathologic Features, Management, and Outcomes Of Pediatric CRTC1::TRIM11 and MED15::ATF1 Tumors With Spitzoid Morphology: A Case Series.
作者信息
Sargen Michael R, Fitzhugh Madeline H, Ko Jennifer S, Close Allison G, Horton Sara C, Malik Faizan, Furtado Larissa V, Bahrami Armita, Seynnaeve Brittani K N, Pappo Alberto S, Liu Vincent, Cheung Kevin, Pack Svetlana D, Xi Liqiang, Raffeld Mark, Lee Ina, Gagan Jeffrey, Aldape Kenneth, Barr Frederic G, Hewitt Stephen M, Ferrone Christina K, Hayden Anna, Hatton Jessica N, Goldstein Alisa M, Pfeiffer Ruth M, Widemann Brigitte, Malone Mary Frances Wedekind, Gross Andrea M, Kaplan Rosandra, Yohe Marielle E, Berrebi Kristen G
机构信息
Division of Cancer Epidemiology and Genetics, National Cancer Institute, National Institutes of Health, Rockville, Maryland, USA.
Department of Dermatology, University of Iowa Hospitals and Clinics, Iowa City, Iowa, USA.
出版信息
Pediatr Blood Cancer. 2025 Aug;72(8):e31814. doi: 10.1002/pbc.31814. Epub 2025 May 19.
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.
关于儿童CRTC1::TRIM11（CTRTs）和MED15::ATF1重排肿瘤（MARTs）的临床病理特征及治疗，目前可用信息有限。在这一系列儿童CTRTs（n = 5）和MARTs（n = 2）中，所有肿瘤均表现为梭形细胞形态并表达黑素细胞标志物。1例CTRT和2例MART发生了转移进展：所有3例均接受了免疫治疗，2例患者病情得到控制，1例因疾病进展死亡。第四例患者有淋巴结微转移，病情稳定未进展。该病例系列突出了这些肿瘤潜在的侵袭性，并提示免疫治疗可能是晚期疾病的有效治疗选择。
Zotero 插件