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Abstract：
BACKGROUND: Anaplastic lymphoma kinase (ALK)-positive histiocytosis (ALK-H) is an emerging entity in the category of histiocytic neoplasms that was first reported as a multisystemic disease in three infants in 2008. The clinicopathological spectrum of ALK-H has been expanded to include localized disorders in specific organs, but the features of this subtype are not well known. The authors report a case of ALK-H localized in the central nervous system that was difficult to treat and review the relevant literature.
METHODS: The authors reviewed archival histiocytic tumors at their institute and found a pediatric case of ALK-H localized in a cerebellar hemisphere that had previously been reported as histiocytic sarcoma. Chemotherapy (approximately 1 year), additional surgery, and conventional chemotherapy (approximately 2.5 years) led to clinical remission, and maintenance chemotherapy was continued (approximately 1.5 years). Three years after completing treatment, a high-grade glioma was found in a cerebral hemisphere, and the patient died of the glioma 2 years later.
CONCLUSIONS: Although the prognosis of ALK-H is generally good according to prior cases, the authors\' case required long-term conventional chemotherapy, suggesting the tumor displayed aggressive characteristics. Early administration of ALK inhibitors may be necessary.
METHODS: The authors reviewed archival histiocytic tumors at their institute and found a pediatric case of ALK-H localized in a cerebellar hemisphere that had previously been reported as histiocytic sarcoma. Chemotherapy (approximately 1 year), additional surgery, and conventional chemotherapy (approximately 2.5 years) led to clinical remission, and maintenance chemotherapy was continued (approximately 1.5 years). Three years after completing treatment, a high-grade glioma was found in a cerebral hemisphere, and the patient died of the glioma 2 years later.
CONCLUSIONS: Although the prognosis of ALK-H is generally good according to prior cases, the authors\' case required long-term conventional chemotherapy, suggesting the tumor displayed aggressive characteristics. Early administration of ALK inhibitors may be necessary.
摘要：
背景：间变性淋巴瘤激酶（ALK）阳性组织细胞增生症（ALK-H）是组织细胞肿瘤类别中的新兴实体，2008年首次报道为3名婴儿的多系统疾病。ALK-H的临床病理范围已扩展到包括特定器官的局部疾病，但这种亚型的特征并不为人所知。作者报告1例位于中枢神经系统的ALK-H难以治疗，并复习相关文献。
方法：作者回顾了他们研究所的存档组织细胞肿瘤，发现了一例位于小脑半球的ALK-H患儿病例，以前曾报道过这种病例为组织细胞肉瘤。化疗（约1年），额外的手术,和常规化疗（大约2.5年）导致临床缓解，并继续维持化疗(约1.5年).完成治疗三年后，在大脑半球发现了一个高级别神经胶质瘤，2年后患者死于胶质瘤.
结论：尽管根据以前的病例，ALK-H的预后通常良好，作者\'病例需要长期常规化疗，提示肿瘤表现出侵袭性特征。ALK抑制剂的早期给药可能是必要的。
方法：作者回顾了他们研究所的存档组织细胞肿瘤，发现了一例位于小脑半球的ALK-H患儿病例，以前曾报道过这种病例为组织细胞肉瘤。化疗（约1年），额外的手术,和常规化疗（大约2.5年）导致临床缓解，并继续维持化疗(约1.5年).完成治疗三年后，在大脑半球发现了一个高级别神经胶质瘤，2年后患者死于胶质瘤.
结论：尽管根据以前的病例，ALK-H的预后通常良好，作者\'病例需要长期常规化疗，提示肿瘤表现出侵袭性特征。ALK抑制剂的早期给药可能是必要的。
