Power Phoebe, Payne Susannah, Walsh Rebecca, Nelson Adam, Manoharan Neevika
Kids Cancer Centre, Sydney Children's Hospital, High Street, Randwick, NSW, 2031, Australia.
Department of Pediatric Oncology, Dana-Farber/Boston Children's Cancer and Blood Disorders Center, Boston, MA, USA.
Childs Nerv Syst. 2025 May 29;41(1):192. doi: 10.1007/s00381-025-06855-9.
Myeloid neoplasms-post cytotoxic therapy (MN-pCT, previously therapy-related myeloid neoplasms/tMN), are secondary malignancies associated with prior chemotherapy treatment, historically carrying a very poor prognosis. These are rarely associated with primary central nervous system (CNS) tumors, usually high-grade CNS malignancies requiring intensive multimodal treatment. Pediatric low-grade gliomas (pLGGs) are the most common childhood CNS tumors, and up to 50% of patients will require adjuvant therapy, which has traditionally consisted of low-dose metronomic chemotherapy, though the recent identification of key molecular drivers of pLGG means targeted therapies are changing this paradigm. We present a novel case of a 17-year-old girl with therapy-related myelodysplastic syndrome following chemotherapeutic treatment for pLGG. Given the poor prognosis of MN-pCTs, this case represents an important note of caution when choosing appropriate therapy for pLGG, especially considering the evolving role for targeted treatments in this disease.
髓系肿瘤-细胞毒性治疗后（MN-pCT，以前称为治疗相关髓系肿瘤/tMN），是与先前化疗相关的继发性恶性肿瘤，历来预后很差。这些肿瘤很少与原发性中枢神经系统（CNS）肿瘤相关，原发性中枢神经系统肿瘤通常是需要强化多模式治疗的高级别中枢神经系统恶性肿瘤。小儿低级别胶质瘤（pLGG）是儿童最常见的中枢神经系统肿瘤，高达50%的患者需要辅助治疗，传统上辅助治疗包括低剂量节拍化疗，不过最近对pLGG关键分子驱动因素的鉴定意味着靶向治疗正在改变这一模式。我们报告了一例17岁女孩的新病例，该女孩在接受pLGG化疗后发生了治疗相关的骨髓增生异常综合征。鉴于MN-pCT的预后较差，该病例为选择pLGG的合适治疗方法时提供了重要的警示，尤其是考虑到靶向治疗在该疾病中不断演变的作用。
