Cacciotti Chantel, Chordas Christine, Valentino Katie, Allen Rudy, Lenzen Alicia, Burns Karen, Nagarajan Rajaram, Manley Peter, Pillay-Smiley Natasha
Dana Farber/Boston Children's Cancer and Blood Disorders Center, Boston, Massachusetts, USA.
Ann & Robert H. Lurie Children's Hospital, Northwestern University, Chicago, Illinois, USA.
Neurooncol Pract. 2022 Apr 23;9(4):338-343. doi: 10.1093/nop/npac030. eCollection 2022 Aug.
Medulloblastoma is an aggressive central nervous system (CNS) tumor that occurs mostly in the pediatric population. Treatment often includes a combination of surgical resection, craniospinal irradiation (CSI), and chemotherapy. Children who receive standard photon CSI are at risk for cardiac toxicities including coronary artery disease, left ventricular scarring and dysfunction, valvular damage, and atherosclerosis. Current survivorship guidelines recommend routine echocardiogram (ECHO) surveillance. In this multi-institutional study, we describe markers of cardiac dysfunction in medulloblastoma survivors.
A retrospective chart review of medulloblastoma patients who had photon beam CSI was followed by ECHO between 1980 and 2010 at Lurie Children's Hospital and Dana-Farber/Boston Children's Hospital.
During the 30-year study period, 168 medulloblastoma patient records were identified. Included in this study were the 75 patients who received CSI or spinal radiation and ECHO follow-up. The mean age at CSI was 8.6 years (range, 2.9-20), and the mean number of years between radiation therapy (RT) completion and first ECHO was 7.4 (range, 2-16). Mean ejection fraction (EF) was 60.0% and shortening fraction (SF) was 33.8%. Five patients (7%) had abnormal ECHO results: three with EF <50% and two with SF <28%.
The majority of medulloblastoma patients who received CSI have relatively normal ECHOs post-treatment; however, 7% of patients had abnormal ECHOs. The implication of our study for medulloblastoma survivors is that further investigations are needed in this population with a more systematic, longitudinal assessment to determine predictors and screenings.
髓母细胞瘤是一种侵袭性中枢神经系统（CNS）肿瘤，主要发生于儿童群体。治疗通常包括手术切除、全脑全脊髓放疗（CSI）和化疗。接受标准光子CSI治疗的儿童存在心脏毒性风险，包括冠状动脉疾病、左心室瘢痕形成和功能障碍、瓣膜损害以及动脉粥样硬化。目前的生存指南建议进行常规超声心动图（ECHO）监测。在这项多机构研究中，我们描述了髓母细胞瘤幸存者心脏功能障碍的标志物。
对1980年至2010年间在卢里儿童医院和达纳-法伯/波士顿儿童医院接受光子束CSI治疗的髓母细胞瘤患者进行回顾性病历审查，并随后进行ECHO检查。
在30年的研究期间，共识别出168例髓母细胞瘤患者记录。本研究纳入了75例接受CSI或脊髓放疗并进行ECHO随访的患者。CSI时的平均年龄为8.6岁（范围2.9 - 20岁），放疗（RT）结束至首次ECHO检查的平均年数为7.4年（范围2 - 16年）。平均射血分数（EF）为60.0%，缩短分数（SF）为33.8%。5例患者（7%）ECHO结果异常：3例EF <50%，2例SF <28%。
大多数接受CSI治疗的髓母细胞瘤患者治疗后ECHO结果相对正常；然而，7%的患者ECHO结果异常。我们的研究对髓母细胞瘤幸存者的意义在于，需要对该人群进行更系统、纵向的评估，以确定预测因素并进行筛查。
