Department of Neurosurgery, Hadassah-Hebrew University Medical Center, Jerusalem, Israel.
Spine (Phila Pa 1976). 2011 Nov 1;36(23):E1534-9. doi: 10.1097/BRS.0b013e31822dddf4.
Review the presentation and diagnosis of an intradural extramedullary hemangiopericytoma of the thoracic spine.
To present a rare case of intradural, subpial hemangiopericytoma in the thoracic spine, with a brief overview of the literature.
Spinal intradural extramedullary hemangiopericytoma is rare entity that radiographically mimics nerve-sheath tumors. These lesions are typically diagnosed at surgery performed due to suspicion of tumor.
A 20-year-old man who presented with back pain, leg weakness, and sphincter incontinence. MR imaging demonstrated an intradural extramedullary lesion at the T9-T10 level that was isointense on T1- and T2-weighted images and homogeneously enhancing after administration of gadolinium, with cystic components seen on T2-weighted images. The preoperative diagnosis was meningioma or schwannoma.
At surgery, the lesion was bluish and completely subpial, with apparent nerve root invasion. Pathological examination revealed a neoplasm adjacent to a nerve root with possible focal infiltration. Abundant reticulin fibers and widened, branching vascular channels imparting a staghorn appearance were seen. Up to five mitotic figures were counted in one high-power field. On immunostaining, the neoplastic cells were diffusely immunoreactive for CD99 and immunonegative for EMA, CD34, and S-100 protein. The pathological diagnosis was consistent with anaplastic hemangiopericytoma, WHO grade III.
This is the ninth report of spinal intradural hemangiopericytoma. The location of the neoplasm supports the hypothesis that hemangiopericytoma may arise from the spinal pial capillaries.
回顾一例胸椎管内硬脊膜外血管外皮细胞瘤的表现和诊断。
介绍一例罕见的胸椎管内、软膜下血管外皮细胞瘤，并简要回顾文献。
脊髓硬脊膜外髓外血管外皮细胞瘤是一种罕见的实体，在影像学上类似于神经鞘瘤。这些病变通常在因怀疑肿瘤而进行的手术中诊断。
一名 20 岁男性，表现为背痛、下肢无力和括约肌失禁。MR 成像显示 T9-T10 水平的硬脊膜外髓外病变，在 T1 和 T2 加权图像上呈等信号，在注射钆后均匀增强，在 T2 加权图像上可见囊性成分。术前诊断为脑膜瘤或神经鞘瘤。
手术时，病变呈蓝色，完全位于软膜下，明显侵犯神经根。病理检查显示靠近神经根的肿瘤可能有局灶性浸润。大量网状纤维和增宽、分支的血管通道，呈鹿角状外观。在一个高倍视野中最多可计数到 5 个有丝分裂象。免疫染色显示，肿瘤细胞弥漫性表达 CD99，免疫阴性表达 EMA、CD34 和 S-100 蛋白。病理诊断为间变型血管外皮细胞瘤，WHO 分级 III 级。
这是第九例报告的脊髓硬脊膜内血管外皮细胞瘤。肿瘤的位置支持血管外皮细胞瘤可能起源于脊髓软膜毛细血管的假说。
