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Malignant Triton Tumor in a Child: Case Report and Literature Review

机译：儿童的恶性Triton肿瘤：案例报告和文献综述

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Objective: Malignant triton tumor (MTT) is a rare and devastating malignant peripheral nerve-sheath tumor, which shows rapid growth and poor clinical outcomes. Here, we reported a 2-year-old girl who was diagnosed as MTT, an overview of the literature was conducted to discuss the clinical features and optimal treatment strategies of MTT. Methods: We conducted an analysis of 42 patients from the PubMed, Medline, Embase and Web of Science databases for relevant articles published between 1938 and 2018. Results: A 2-year-old girl died of tumor recurrence. Forty-two eligible cases of MTT in children (birth to 18 years; mean age, 8.3 years), the highest frequency of occurrence was in 12–16 years; and the male-to-female ratio was 1.7:1. Only 33 provided complete treatment details: 11 patients received treatment by surgery alone; 2 received both surgery and chemotherapy; 4 received both surgery and radiation therapy; 14 received surgery, chemotherapy, and radiation therapy; 1 case received chemotherapy and radiation therapy; and only 1 received supportive care. In the 33 cases, the average OS and 5-year OS probability were 23.9 months (range, 0.3–156 months) and 27.5 ± 4.3%. There were significant differences between radiation therapy and patient survival (p0.05), postoperative chemotherapy/radiation therapy and patient prognosis (p0.05). Conclusion: The clinical and histopathological features and therapeutic options for MTT are discussed in the light of published data. Further studies are needed to improve survival in children with this rare malignant tumor.
机译：目的：恶性氚核肿瘤（MTT）是一种难得和破坏性的恶性周围神经鞘瘤，其展示了快速增长和临床结果不佳。在这里，我们报告了一名2岁的女孩被诊断为MTT，概述了文献的概述，讨论了MTT的临床特征和最佳治疗策略。方法：对1938年至2018年间公布的有关文章的PubMed，Medline，Embase和Science数据库网站的42名患者进行了分析。结果：一名2岁的女孩死于肿瘤复发。儿童的42例符合条件的MTT案例（出生18岁;平均年龄，8.3岁），最高频率发生在12-16岁;雄性与女性比例为1.7：1。只有33条提供了完整的治疗细节：11名患者单独接受手术治疗; 2接受手术和化疗; 4接受手术和放射治疗; 14接受手术，化疗和放射治疗; 1例接受化学疗法和放射治疗;而且只有1个支持的护理。在33例中，平均OS和5年的OS概率为23.9个月（范围，0.3-156个月）和27.5±4.3％。放射治疗和患者存活之间存在显着差异（P <0.05），术后化疗/放射治疗和患者预后（P <0.05）。结论：根据公布数据讨论了MTT的临床和组织病理学特征和治疗选择。需要进一步的研究来改善具有这种罕见的恶性肿瘤儿童的生存。

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