Department of Neurological Surgery, University of California, Davis, California, USA; Chicago Medical School, Rosalind Franklin University of Medicine and Science, North Chicago, USA.
Department of Neurological Surgery, University of California, Davis, California, USA.
World Neurosurg. 2021 Apr;148:29-37. doi: 10.1016/j.wneu.2021.01.004. Epub 2021 Jan 11.
Intracranial myeloid sarcomas (IMS) are rare central nervous system manifestations of malignant hematopoietic neoplasms of myeloid origin such as acute myeloid leukemia and chronic myeloid leukemia. Reported cases in the literature are limited to primarily case reports. We present a systematic review of this rare central nervous system tumor, characterizing the clinical presentation, tumor location, histopathology, and available treatment modalities. We correlate these variables with mortality, recurrence, and complications to suggest optimal management strategies for IMS.
A systematic literature search was performed across Ovid MEDLINE, Scopus, and Embase using 14 search terms in accordance to Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. This systematic review examines variables such as patient age, tumor location, size, presenting symptoms, treatment modality, extent of resection, and mortality. We performed descriptive analyses to identify bivariate associations between patient characteristics, treatment, and outcomes.
The mean age at diagnosis was 34.8 years, and the most common etiology was acute myeloid leukemia (68.8%). The most common presenting symptoms were headache (45.5%), vision complaints (27.3%), and weakness/motor symptoms (21.2%). IMS were most commonly located in the temporal lobe (10.1%), cerebellum (10.1%), or falcine/parasagittal (10.1%) region. Patients who received radiotherapy (P < 0.001) or chemotherapy (P < 0.001) had lower rates of mortality versus those who did not. Surgical treatment and extent of resection were not significantly associated with mortality (P > 0.05).
The use of adjuvant radiotherapy or chemotherapy for IMS significantly reduces mortality, confirming IMS as a cranial manifestation of a systemic disease. Although surgical treatment is indicated for histopathologic diagnosis and to relieve mass effect, the extent of resection does not predict overall survival.
颅内髓样肉瘤（IMS）是髓系恶性造血肿瘤如急性髓系白血病和慢性髓系白血病等罕见的中枢神经系统表现。文献中的报道病例主要为个案报告。我们对这种罕见的中枢神经系统肿瘤进行了系统综述，描述了其临床表现、肿瘤位置、组织病理学和可用的治疗方式。我们将这些变量与死亡率、复发和并发症相关联，以提出 IMS 的最佳管理策略。
根据系统评价和荟萃分析的首选报告项目，通过 Ovid MEDLINE、Scopus 和 Embase 进行了系统的文献检索，使用了 14 个搜索词。本系统综述检查了患者年龄、肿瘤位置、大小、首发症状、治疗方式、切除范围和死亡率等变量。我们进行了描述性分析，以确定患者特征、治疗和结果之间的双变量关联。
诊断时的平均年龄为 34.8 岁，最常见的病因是急性髓系白血病（68.8%）。最常见的首发症状是头痛（45.5%）、视力障碍（27.3%）和无力/运动症状（21.2%）。IMS 最常位于颞叶（10.1%）、小脑（10.1%）或镰旁/矢状旁（10.1%）区域。接受放疗（P<0.001）或化疗（P<0.001）的患者死亡率低于未接受治疗的患者。手术治疗和切除范围与死亡率无显著相关性（P>0.05）。
对 IMS 采用辅助放疗或化疗可显著降低死亡率，证实 IMS 是全身性疾病的颅部表现。尽管手术治疗是为了组织病理学诊断和缓解肿块效应，但切除范围并不能预测总体生存率。
