Nishii Tomoya, Nagashima Yoshitaka, Nishimura Yusuke, Ito Hiroshi, Oyama Takahiro, Matsuo Mamoru, Sakakibara Ayako, Shimada Satoko, Saito Ryuta
Department of Neurosurgery, Nagoya University, Nagoya, Japan.
Department of Pathology, Nagoya University, Nagoya, Japan.
J Spine Surg. 2021 Dec;7(4):532-539. doi: 10.21037/jss-21-83.
Solitary fibrous tumors (SFTs) and hemangiopericytomas (HPCs) have been classified as one entity by the World Health Organization in 2016 due to gene fusion between NAB2 and STAT6. In the Central Nervous System (CNS), a hypocellular, collagenized tumor with a classic SFT phenotype is considered grade I, whereas more densely cellular tumors mostly corresponding to the HPC phenotype are classified as grade II or III (anaplastic) depending in mitotic count (<5 >5 mitoses per 10 high-power fields). Herein, we report two cases of targeted SFT/HPC in which pathological differences and WHO grading affect clinical features. A 75-year-old woman presented with headache and had an intradural extramedullary tumor at the C1 to C2 level. The tumor was well-circumscribed and attached only to the dura mater. It was totally removed and diagnosed SFT/HPC grade I. In contrast, a 68-year-old woman presented with numbness in the right upper limb and had an intradural extramedullary tumor at the medulla to C3 levels The tumor was irregularly marginated and strongly adherent to the spinal cord and involved the vertebral artery. It was sub totally removed and diagnosed SFT/HPC grade II. To the best of our knowledge, there are only 12 cases of SFT/HPC at the craniocervical junction, including the present two cases, of which four that were adherent to the spinal cord or involved the vertebral artery were grade II or III. Although the location of the tumor was almost the same, there were significant differences in the intraoperative findings according to the WHO grading.
2016年，由于NAB2和STAT6之间的基因融合，孤立性纤维性肿瘤（SFTs）和血管外皮细胞瘤（HPCs）被世界卫生组织归为同一实体。在中枢神经系统（CNS）中，具有典型SFT表型的细胞稀少、胶原化的肿瘤被认为是I级，而细胞密度更高、大多对应HPC表型的肿瘤则根据有丝分裂计数（每10个高倍视野中<5个或>5个有丝分裂）被分类为II级或III级（间变性）。在此，我们报告两例靶向性SFT/HPC病例，其中病理差异和世界卫生组织分级影响临床特征。一名75岁女性因头痛就诊，在C1至C2水平有硬膜内髓外肿瘤。肿瘤边界清晰，仅附着于硬脑膜。肿瘤被完全切除，诊断为I级SFT/HPC。相比之下，一名68岁女性因右上肢麻木就诊，在延髓至C3水平有硬膜内髓外肿瘤。肿瘤边界不规则，与脊髓紧密粘连并累及椎动脉。肿瘤次全切除，诊断为II级SFT/HPC。据我们所知，颅颈交界区的SFT/HPC病例仅有12例，包括本两例，其中四例与脊髓粘连或累及椎动脉的为II级或III级。尽管肿瘤位置几乎相同，但根据世界卫生组织分级，术中发现存在显著差异。
