Anaplastic Lymphoma Kinase (ALK) has been implicated in several human cancers. This review aims at mapping the available literature on the involvement of ALK in non-glial tumors localized in the posterior cranial fossa and at identifying diagnostic, prognostic, and therapeutic considerations. Following the PRISMA-ScR guidelines, studies were included if they investigated ALK’s role in primary CNS, non-glial tumors located in the posterior cranial fossa. A total of 210 manuscripts were selected for full-text review and 16 finally met the inclusion criteria. The review included 55 cases of primary, intracranial neoplasms with ALK genetic alterations and/or protein expression, located in the posterior fossa, comprising of medulloblastoma, anaplastic large-cell lymphoma, histiocytosis, inflammatory myofibroblastic tumors, and intracranial myxoid mesenchymal tumors. ALK pathology was investigated via immunohistochemistry or genetic analysis. Several studies provided evidence for potential diagnostic and prognostic value for ALK assessment as well as therapeutic efficacy in its targeting. The available findings on ALK in posterior fossa tumors are limited. Nevertheless, previous findings suggest that ALK assessment is of diagnostic and prognostic value in medulloblastoma (WNT-activated). Interestingly, a substantial proportion of ALK-positive/altered CNS histiocytoses thus far identified have been localized in the posterior fossa. The therapeutic potential of ALK inhibition in histiocytosis warrants further investigation.
间变性淋巴瘤激酶(ALK)与多种人类癌症的发生相关。本综述旨在梳理现有关于ALK在后颅窝非胶质肿瘤中作用的文献,并明确其诊断、预后及治疗意义。依据PRISMA-ScR指南,本研究纳入所有探讨ALK在后颅窝原发性中枢神经系统非胶质肿瘤中作用的研究。经筛选共210篇文献进入全文评审阶段,最终16篇符合纳入标准。综述共涵盖55例位于后颅窝、存在ALK基因改变和/或蛋白表达的原发性颅内肿瘤病例,包括髓母细胞瘤、间变性大细胞淋巴瘤、组织细胞增生症、炎性肌纤维母细胞瘤及颅内黏液样间叶性肿瘤。ALK病理学特征通过免疫组织化学或基因分析进行研究。多项研究证实ALK检测具有潜在的诊断与预后价值,其靶向治疗亦显示出疗效。目前关于后颅窝肿瘤中ALK的研究数据有限,但既往研究表明ALK检测对WNT激活型髓母细胞瘤具有诊断及预后价值。值得注意的是,目前已发现的ALK阳性/突变型中枢神经系统组织细胞增生症中,有相当比例定位于后颅窝。ALK抑制剂在组织细胞增生症中的治疗潜力值得进一步深入研究。