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脊髓血管外皮细胞瘤:机构经验及文献综述

Spinal hemangiopericytoma: an institutional experience and review of literature.

作者信息

Das Amitabha, Singh Pankaj Kumar, Suri Vaishali, Sable Mukund N, Sharma Bhawani Shankar

机构信息

Department of Neurosurgery, All India Institute of Medical Sciences, New Delhi, India.

出版信息

Eur Spine J. 2015 May;24 Suppl 4:S606-13. doi: 10.1007/s00586-015-3789-1. Epub 2015 Feb 8.

Abstract

PURPOSE

Hemangiopericytoma is a rare tumor of CNS with potential for recurrence and widespread metastasis, even outside CNS with even rare involvement of spinal cord. This case series presents five patients to evaluate the clinical presentation, radiological features, management, pathology and outcome of spinal hemangiopericytomas.

METHODS

Between 2004 and 2013, five patients underwent surgery for spinal hemangiopericytoma. Histopathological data were reviewed in all cases and clinical and follow-up details were collected from the data available in our department.

RESULTS

There were three males and two females, including one pediatric patient. Three patients had dorsal spine involvement and two patients had involvement of cervical spine. There were two patients with intradural extramedullary tumors, one patient each with pure intramedullary tumor, pure extradural tumor and both intra and extradural tumor. All of them presented with motor weakness. Gross total resection of the tumor was done in three patients. Four patients received post-operative radiotherapy. Histopathology showed anaplastic tumor in four cases with high MIB-1 LI. Most of them were positive for CD34, mic-2 and bcl-2. Three patients who underwent gross total resection improved significantly in the follow-up period. Two patients who underwent subtotal resection expired due to spread of their disease.

CONCLUSION

Spinal hemangiopericytoma is a rare tumor. Strong clinical suspicion is required to diagnose it pre-operatively. Gross total resection is the goal and radiotherapy should be given in case of residual tumor or high-grade tumors.

摘要

目的

血管外皮细胞瘤是一种罕见的中枢神经系统肿瘤,有复发和广泛转移的可能,甚至可转移至中枢神经系统以外,脊髓受累更为罕见。本病例系列报告了5例患者,以评估脊髓血管外皮细胞瘤的临床表现、影像学特征、治疗、病理及预后。

方法

2004年至2013年间,5例患者接受了脊髓血管外皮细胞瘤手术。所有病例均回顾了组织病理学数据,并从本部门现有数据中收集了临床及随访细节。

结果

男性3例,女性2例,其中1例为儿童患者。3例患者病变累及胸椎,2例累及颈椎。2例为硬脊膜下髓外肿瘤,1例为单纯髓内肿瘤,1例为单纯硬膜外肿瘤,1例为硬膜内外肿瘤。所有患者均表现为肌无力。3例患者实现了肿瘤全切。4例患者接受了术后放疗。组织病理学显示4例为间变性肿瘤,MIB-1标记指数高。多数病例CD34、mic-2和bcl-2呈阳性。3例接受肿瘤全切的患者在随访期间明显改善。2例接受次全切除的患者因疾病扩散死亡。

结论

脊髓血管外皮细胞瘤是一种罕见肿瘤。术前诊断需要高度的临床怀疑。手术全切是治疗目标,残留肿瘤或高级别肿瘤患者应接受放疗。

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