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

IF 2.4 3区医学 Q2 HEMATOLOGY

Pediatric Blood & Cancer Pub Date : 2025-05-19 DOI:10.1002/pbc.31814

Michael R Sargen, Madeline H Fitzhugh, Jennifer S Ko, Allison G Close, Sara C Horton, Faizan Malik, Larissa V Furtado, Armita Bahrami, Brittani K N Seynnaeve, Alberto S Pappo, Vincent Liu, Kevin Cheung, Svetlana D 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 Widemann, Mary Frances Wedekind Malone, Andrea M Gross, Rosandra Kaplan, Marielle E Yohe, Kristen G Berrebi

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引用次数: 0

Abstract

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.

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儿童CRTC1::TRIM11和MED15::ATF1 Spitzoid形态肿瘤的临床病理特征、处理和结局：一个病例系列

关于儿童CRTC1::TRIM11 （CTRTs）和MED15:: atf1重排肿瘤（MARTs）的临床病理特征和治疗信息有限。在这一系列的儿童ctrt （n = 5）和mart （n = 2）中，所有肿瘤均表现为spitzoid形态并表达黑素细胞标记物。1例CTRT和2例mart发生转移性进展：所有3例患者均接受免疫治疗，2例患者疾病得到控制，1例患者因疾病进展而死亡。第四名患者的淋巴结微转移保持稳定，没有进展。本病例系列强调了这些肿瘤潜在的侵袭性行为，并提示免疫治疗可能是晚期疾病的有效治疗选择。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Pediatric Blood & Cancer 医学-小儿科

CiteScore

4.90

自引率

9.40%

发文量

546

审稿时长

1.5 months

期刊介绍： Pediatric Blood & Cancer publishes the highest quality manuscripts describing basic and clinical investigations of blood disorders and malignant diseases of childhood including diagnosis, treatment, epidemiology, etiology, biology, and molecular and clinical genetics of these diseases as they affect children, adolescents, and young adults. Pediatric Blood & Cancer will also include studies on such treatment options as hematopoietic stem cell transplantation, immunology, and gene therapy.