Background Primary intraspinal hemangiopericytoma is a rare malignant mesenchymal tumor with high rates of recurrence and metastasis. Surgery is the main therapeutic procedure for this lesion. This clinical research w...Background Primary intraspinal hemangiopericytoma is a rare malignant mesenchymal tumor with high rates of recurrence and metastasis. Surgery is the main therapeutic procedure for this lesion. This clinical research was undertaken to analyze the pathological characteristics, clinical course, and the choice of treatment for this lesion. Methods Twenty-three patients with primary intraspinal hemangiopericytomas were treated from 1987 to 2004. The clinical and imaging features, pathological findings, therapeutic procedures, and prognosis were analyzed retrospectively. Results Primary intraspinal hemangiopericytoma is more likely to attack middle-aged persons. The tumor mainly manifests as muscle weakness and sensor abnormalities. Microscopic examination showed slit-like vascular spaces and oral- or spindle-shaped cells with slightly acidic cytoplasm and oral nuclei. Tumors were subtotally resected in 11 patients, subtotally resected with postoperative radiotherapy in 4, totally resected in 5, and totally resected with postoperative radiotherapy in 3. Two patients were given spinal stabilization after total resection. Recurrence and metastatic rates were 50% and 0 in intradural patients. They were 73% and 27% in extradural patients, respectively. Conclusions The tumor should be resected en bloc with the neighboring dural mater to reduce recurrence and metastasis. Patients with subtotal resection need adjuvant radiotherapy. Patients with evident spinal involvement may benefit from spinal stabilization. The prognosis of the lesion arising from the dural mater is better.展开更多
目的由于中枢神经系统（CNS）孤立性纤维性肿瘤（SFT）与血管外皮瘤（HPC）的分子遗传学特征一致，2016 WHO CNS肿瘤分类将二者合为SFT/HPC，并分为3级。本文主要探讨CNS SFT/HPC的临床病理学特征、诊断、鉴别诊断及临床预后等。方法收...目的由于中枢神经系统（CNS）孤立性纤维性肿瘤（SFT）与血管外皮瘤（HPC）的分子遗传学特征一致，2016 WHO CNS肿瘤分类将二者合为SFT/HPC，并分为3级。本文主要探讨CNS SFT/HPC的临床病理学特征、诊断、鉴别诊断及临床预后等。方法收集71例CNS SFT和HPC进行回顾性研究和重新分级，对组织形态学、免疫组织化学和临床影像资料进行分析，并进行随访及复习相关文献。结果71例患者中男性37例，女性34例；年龄3～77岁，中位年龄48岁。WHO Ⅰ级12例（17%）、WHOⅡ级26例（37%）和WHOⅢ级33例（46%）。镜下可表现为经典的SFT表型、HPC表型或二者混合。免疫表型上，97%（69/71）的病例表达STAT6，其中96%（66/69）的病例弥漫强阳性；bcl－2、CD99及波形蛋白表达率均约为90%；CD34表达率随着肿瘤级别升高而降低，平均表达率为78%，10%（7/71）的病例不同程度表达SSTR2a，其中1例胞质强阳性；极少数病例局灶表达上皮细胞膜抗原、CD57、S－100蛋白；Ki－67阳性指数1%～50%。34例随访8～130个月，其中12例复发（35%），2例（6%）发生肝转移。结论CNS SFT/HPC相对少见，不同级别之间临床病理和预后之间明显重叠或过渡。STAT6是目前诊断该肿瘤较为特异的标志物。手术切除为首选治疗手段，Ⅱ级和Ⅲ级易复发及转移，最常转移部位为肝脏。展开更多
文摘Background Primary intraspinal hemangiopericytoma is a rare malignant mesenchymal tumor with high rates of recurrence and metastasis. Surgery is the main therapeutic procedure for this lesion. This clinical research was undertaken to analyze the pathological characteristics, clinical course, and the choice of treatment for this lesion. Methods Twenty-three patients with primary intraspinal hemangiopericytomas were treated from 1987 to 2004. The clinical and imaging features, pathological findings, therapeutic procedures, and prognosis were analyzed retrospectively. Results Primary intraspinal hemangiopericytoma is more likely to attack middle-aged persons. The tumor mainly manifests as muscle weakness and sensor abnormalities. Microscopic examination showed slit-like vascular spaces and oral- or spindle-shaped cells with slightly acidic cytoplasm and oral nuclei. Tumors were subtotally resected in 11 patients, subtotally resected with postoperative radiotherapy in 4, totally resected in 5, and totally resected with postoperative radiotherapy in 3. Two patients were given spinal stabilization after total resection. Recurrence and metastatic rates were 50% and 0 in intradural patients. They were 73% and 27% in extradural patients, respectively. Conclusions The tumor should be resected en bloc with the neighboring dural mater to reduce recurrence and metastasis. Patients with subtotal resection need adjuvant radiotherapy. Patients with evident spinal involvement may benefit from spinal stabilization. The prognosis of the lesion arising from the dural mater is better.
