Department of Neurological Surgery, University of Miami Miller School of Medicine, Lois Pope Life Center, Miami, Florida, USA.
Department of Pathology, University of Miami Miller School of Medicine, Lois Pope Life Center, Miami, Florida, USA.
Neurosurgery. 2021 May 13;88(6):1074-1087. doi: 10.1093/neuros/nyab043.
The intracerebral occurrence of malignant peripheral nerve sheath tumors (MPNSTs) is exceedingly rare, and despite aggressive treatments, local recurrence and poor prognosis are very frequent. Like other brain tumors, these tumors could be primary or secondary, making the term "peripheral" an imprecise term for a primary brain tumor.
To analyze the reported cases of primary and secondary cerebral MPSNTs in terms of diagnosis, treatment, and overall survival. Additionally, we present a case of malignant intracerebral nerve sheath tumor (MINST) treated with radical surgery and radiotherapy.
Using the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines, one database (PubMed) and crossed references were queried for MPNST with brain metastasis and primary MINSTs from 1971 to 2020. Data regarding demographic features, primary tumor site, risk factors, brain location of the lesion, treatment applied, and overall survival were extracted.
A total of 55 patients were selected (including the reported case): 29 patients were secondary brain MPNST and 26 patients were primary MINST. The mean age was 41.8 ± 22 and 31.2 ± 23 yr, respectively. All brain metastases of MPNST (100%) had a primary tumor elsewhere in the body at the time of diagnosis. The overall survival was significantly shorter in patients with a secondary brain MPNST compared to MINST (P = .002).
We present a comprehensive analysis of every reported primary and secondary intracerebral MPNST. The prognosis in terms of survival is worst in the last one despite aggressive treatment. The lack of a primary MPNST in screening tests is sufficient to confirm a MINST at time of diagnosis.
颅内恶性外周神经鞘瘤（MPNST）极为罕见，尽管采用了积极的治疗方法，但局部复发和预后不良仍很常见。与其他脑肿瘤一样，这些肿瘤可能是原发性的，也可能是继发性的，因此“外周”一词对于原发性脑肿瘤来说并不准确。
分析报告的原发性和继发性脑 MPSNT 病例，从诊断、治疗和总体生存方面进行分析。此外，我们还报告了一例经根治性手术和放疗治疗的恶性颅内神经鞘瘤（MINST）病例。
采用系统评价和荟萃分析的首选报告项目（PRISMA）指南，检索 1971 年至 2020 年间的 PubMed 数据库和交叉引用文献，检索脑转移和原发性 MINST 的 MPNST 病例。提取人口统计学特征、原发肿瘤部位、危险因素、病变的脑部位置、应用的治疗方法和总体生存率等数据。
共选择了 55 例患者（包括报告的病例）：29 例为继发性脑 MPNST，26 例为原发性 MINST。平均年龄分别为 41.8±22 岁和 31.2±23 岁。所有 MPNST 的脑转移瘤（100%）在诊断时均在身体其他部位存在原发性肿瘤。继发性脑 MPNST 患者的总体生存率明显短于 MINST 患者（P=0.002）。
我们对每例报告的原发性和继发性颅内 MPNST 进行了全面分析。尽管采用了积极的治疗方法，但在继发性脑 MPNST 患者中，生存预后最差。在筛查试验中缺乏原发性 MPNST 足以在诊断时确定 MINST。
