Department of Radiation Oncology, College of Medicine, University of Florida, Jacksonville, Florida.
Department of Pediatric Oncology, Nemours Children's Health Systems, Jacksonville, Florida.
Pediatr Blood Cancer. 2020 Feb;67(2):e28080. doi: 10.1002/pbc.28080. Epub 2019 Nov 17.
Despite the dosimetric advantages of proton therapy, little data exist on patients who receive proton therapy for Ewing sarcoma of the cranium and skull base. This study reports local disease control and toxicity in such patients.
MATERIALS/METHODS: We reviewed 25 patients (≤21 years old) with nonmetastatic Ewing sarcoma of the cranium and skull base treated between 2008 and 2018. Treatment toxicity was graded per the Common Terminology Criteria for Adverse Events v4.0. The Kaplan-Meier product limit method provided estimates of disease control and survival.
Median patient age was 5.9 years (range, 1-21.7). Tumor subsites included the skull base (48%), non-skull-base calvarial bones (28%), paranasal sinuses (20%), and nasal cavity (4%). All patients underwent multiagent alkylator- and anthracycline-based chemotherapy; 16% underwent gross total resection (GTR) before radiation. Clinical target volume (CTV) 1 received 45 GyRBE and CTV2 received 50.4 GyRBE following GTR or 54-55.8 GyRBE following biopsy or subtotal resection. Median follow-up was 3.7 years (range, 0.26-8.3); no patients were lost. The 4-year local control, disease-free survival, and overall survival rates were 96%, 86%, and 92%, respectively. Two patients experienced in-field recurrences. One patient experienced bilateral conductive hearing loss requiring aids, two patients developed intracranial vasculopathy, and 6 patients required hormone replacement therapy for neuroendocrine deficits. None developed a secondary malignancy.
Proton therapy is associated with a favorable therapeutic ratio in children with large Ewing tumors of the cranium and skull base. Despite its high conformality, we observed excellent local control and no marginal recurrences. Treatment dosimetry predicts limited long-term neurocognitive and neuroendocrine side effects.
尽管质子治疗具有剂量学优势，但针对颅底和颅盖骨尤文肉瘤接受质子治疗的患者，相关数据资料较少。本研究报告了此类患者的局部疾病控制和毒性情况。
材料/方法：我们回顾了 2008 年至 2018 年间 25 例（≤21 岁）非转移性颅底和颅盖骨尤文肉瘤患者。按照通用不良事件术语标准 4.0 版对治疗毒性进行分级。Kaplan-Meier 乘积限法提供了疾病控制和生存的估计值。
患者中位年龄为 5.9 岁（范围为 1-21.7 岁）。肿瘤部位包括颅底（48%）、非颅盖骨颅骨（28%）、副鼻窦（20%）和鼻腔（4%）。所有患者均接受多药烷化剂和蒽环类药物为基础的化疗；16%的患者在放疗前行大体全切除（GTR）。GTR 后临床靶区（CTV）1 接受 45 GyRBE，CTV2 接受 50.4 GyRBE；活检或次全切除后，CTV2 接受 54-55.8 GyRBE。中位随访时间为 3.7 年（范围为 0.26-8.3 年）；无失访患者。4 年局部控制、无病生存和总生存率分别为 96%、86%和 92%。2 例患者出现局部区域复发。1 例患者出现双侧传导性听力损失，需要佩戴助听器，2 例患者发生颅内血管病变，6 例患者因神经内分泌缺陷需要激素替代治疗。无患者发生第二恶性肿瘤。
质子治疗在儿童颅底和颅盖骨大尤文瘤患者中具有良好的治疗效果。尽管其适形度高，但我们观察到局部控制良好，无边缘复发。治疗剂量预测长期神经认知和神经内分泌副作用有限。
