Kushel' Iu V, Belova Iu D
FGBU "NII neĭrokhirurgii im. akad. N.N. Burdenko" RAMN, Moskva.
Zh Vopr Neirokhir Im N N Burdenko. 2014;78(6):37-42. doi: 10.17116/neiro201478637-42.
Intramedullary spinal cord tumors (IMSCTs) are a rare neurosurgical disease. There are a lot of controversies in their epidemiology. Most references were published in the 80-90ss. The authors describe and analyze the largest contemporary clinical series of IMSCTs treated by one surgeon (YK).
201 patients (aged 2 months - 18 years) with IMSCT operated on during the past 12 years.
A slight predominance of male patients was discovered. Astrocytomas account for 74% of all pediatric IMSCTs. Classic ependymomas are rare (5,5%). Pediatric spinal cord hemangioblastomas (HABs) are highly suspicious for von Hippel-Lindau disease.
Benign histological variants of astrocytomas are the most common type of pediatric IMCTs. No gender or age differences in the incidence of most tumors (except for HABs) have been observed. Patients with intramedullary HAB should be routinely screened for von Hippel-Lindau disease. There are still a lot of uncertainties in the published data on epidemiology of IMSCTs.
脊髓髓内肿瘤（IMSCTs）是一种罕见的神经外科疾病。其流行病学存在诸多争议。大多数参考文献发表于20世纪80 - 90年代。作者描述并分析了由一位外科医生（YK）治疗的最大规模的当代IMSCTs临床系列病例。
在过去12年中对201例（年龄2个月至18岁）患有IMSCT的患者进行了手术。
发现男性患者略占优势。星形细胞瘤占所有儿童IMSCTs的74%。典型室管膜瘤罕见（5.5%）。儿童脊髓血管母细胞瘤（HABs）高度怀疑与冯·希佩尔 - 林道病有关。
星形细胞瘤的良性组织学变异型是儿童IMCTs最常见的类型。大多数肿瘤（除HABs外）的发病率未观察到性别或年龄差异。患有脊髓内HAB的患者应常规筛查冯·希佩尔 - 林道病。关于IMSCTs流行病学的已发表数据仍存在许多不确定性。
