中枢神经系统孤立性纤维性肿瘤/血管周细胞瘤影像诊断与病理分析
|
摘要
目的探讨中枢神经系统孤立性纤维性肿瘤/血管周细胞瘤的临床影像与病理特点,提高对本病的认识。方法对17例中枢神经系统SFT/HPC进行临床、影像及病理资料分析。结果 17例患者,CT及MRI均显示肿瘤呈结节状、圆形或者椭圆形,16例有宽基底与硬脑膜相连,肿瘤内密度多不均匀,部分伴有出血、囊性变及瘤周水肿。镜下肿瘤细胞呈椭圆形至梭形,染色质较均匀细腻,核仁多不明显,瘤细胞围绕"鹿角状"血管排列。WHOⅡ级12例,Ⅲ级5例。免疫表型:肿瘤细胞弥漫表达vimentin和STAT6,大部分表达CD34、BCL2和CD99。结论中枢神经系统SFT/HPC是一种少见的非脑膜上皮源性肿瘤,临床及影像与脑膜瘤鉴别困难,术后易复发。
Objective To study the imaging and clinicopathological features of solitary fibrous tumor/hemangiopericytoma in the central nervous system in order to improve the knowledge and diagnostic accuracy of this disease. Methods We analyzed the imaging and clinicopathological features of 17 patients who were diagnosed with SFT/HPC of central nervous system. Results In 17 patients, 7 cases were female, 10 weere with male, with mean age of 42 years(from 8 to 75). There were 6 cases in the frontal region, 4 in the temporal region, 2 in the parietal lobe, 2 in the cerebral falx, and 1 in the occipital, cerebellar and cerebellar tentorium respectively. The main clinical manifestations were headache, dizziness, visual impairment, and vomiting. CT and MRI showed that the tumors were nodular, round or oval, and had a wide base connected to the dura mater(16 cases). The density of the tumors were uneven, and some of them were accompanied by hemorrhage, cystic degeneration and peritumoral edema. The tumor cells were elliptical and spindle shape with uniform and delicate chromatin. The nucleolus was not obvious. In some cases, the tumor cells revolved around the "deer horn like" blood vessel. 12 cases were WHO grade Ⅱ, and 5 cases were WHO grade Ⅲ with high cell density, karyoplasmic ratio increased and mitotic activity. Immunophenotype was follows: tumor cells expressed vimentin and STAT-6 diffusely, most of which expressed CD34, BCL2 and CD99. Conclusion SFT/HPC is a rare tumor of non-meningeal epithelia, which is difficult to differentiate from meningioma in clinical and imaging features, and easy to recur after operation.
Objective To study the imaging and clinicopathological features of solitary fibrous tumor/hemangiopericytoma in the central nervous system in order to improve the knowledge and diagnostic accuracy of this disease. Methods We analyzed the imaging and clinicopathological features of 17 patients who were diagnosed with SFT/HPC of central nervous system. Results In 17 patients, 7 cases were female, 10 weere with male, with mean age of 42 years(from 8 to 75). There were 6 cases in the frontal region, 4 in the temporal region, 2 in the parietal lobe, 2 in the cerebral falx, and 1 in the occipital, cerebellar and cerebellar tentorium respectively. The main clinical manifestations were headache, dizziness, visual impairment, and vomiting. CT and MRI showed that the tumors were nodular, round or oval, and had a wide base connected to the dura mater(16 cases). The density of the tumors were uneven, and some of them were accompanied by hemorrhage, cystic degeneration and peritumoral edema. The tumor cells were elliptical and spindle shape with uniform and delicate chromatin. The nucleolus was not obvious. In some cases, the tumor cells revolved around the "deer horn like" blood vessel. 12 cases were WHO grade Ⅱ, and 5 cases were WHO grade Ⅲ with high cell density, karyoplasmic ratio increased and mitotic activity. Immunophenotype was follows: tumor cells expressed vimentin and STAT-6 diffusely, most of which expressed CD34, BCL2 and CD99. Conclusion SFT/HPC is a rare tumor of non-meningeal epithelia, which is difficult to differentiate from meningioma in clinical and imaging features, and easy to recur after operation.
引文
[1] Yuzawa S,Nishihara H,Wang L,et al.Analysis of NAB2-STAT6 gene fusion in 17 cases of meningeal solitary fibrous tumor/hemangiopericytoma review of the literature [J].Am J Surg Pathol,2016,40(8):1031-1040.
[2] Fletcher CD,Bridge JA,Hogendoorn P,et al.WHO classification of tumours of soft tissue and bone[M].Lyon:IARC Press,2013.305-310.
[3] Ecker RD,Marsh WR,Pollock BE,et al.Hemangiopericytoma in the central nervous system:treatment,pathological features and long-term follow up in 38 patients[J].J Neurosurg,2003,98(6):1182-1187.
[4] Marurya J,Seki Y,Morita K,et al.Meningeal hemangiopericytoma manifesting as massive intracranial hemorrhage-two case reports [J].Neurol Med Chir,2006,46(2):92-97.
[5] 杨文圣,季天海,戚沛霖.中枢神经系统血管周细胞瘤1例[J].中国肿瘤临床,2010,37(20):1199.
[6] Champeaux C,Khan AA,Wilson E,et al.Meningeal haemangiopericytoma and solitary fibrous tumour:a retrospective bi centre study for outcome and prognostic factor assessment [J].J Neurooncol,2017,134(2):387-395.
[7] Wei G,Kang X,Liu X,et al.Intracranial meningeal hemangiopericytoma:recurrences at the initial and distant intracranial sites and extraneural metastases to multiple organs [J].Mol Clin Oncol,2015,3(4):770-774.
[2] Fletcher CD,Bridge JA,Hogendoorn P,et al.WHO classification of tumours of soft tissue and bone[M].Lyon:IARC Press,2013.305-310.
[3] Ecker RD,Marsh WR,Pollock BE,et al.Hemangiopericytoma in the central nervous system:treatment,pathological features and long-term follow up in 38 patients[J].J Neurosurg,2003,98(6):1182-1187.
[4] Marurya J,Seki Y,Morita K,et al.Meningeal hemangiopericytoma manifesting as massive intracranial hemorrhage-two case reports [J].Neurol Med Chir,2006,46(2):92-97.
[5] 杨文圣,季天海,戚沛霖.中枢神经系统血管周细胞瘤1例[J].中国肿瘤临床,2010,37(20):1199.
[6] Champeaux C,Khan AA,Wilson E,et al.Meningeal haemangiopericytoma and solitary fibrous tumour:a retrospective bi centre study for outcome and prognostic factor assessment [J].J Neurooncol,2017,134(2):387-395.
[7] Wei G,Kang X,Liu X,et al.Intracranial meningeal hemangiopericytoma:recurrences at the initial and distant intracranial sites and extraneural metastases to multiple organs [J].Mol Clin Oncol,2015,3(4):770-774.