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10 例椎管内血管外皮细胞瘤/孤立性纤维瘤的回顾性研究。

An institutional review of 10 cases of spinal hemangiopericytoma/solitary fibrous tumor.

机构信息

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

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

出版信息

Neurol India. 2020 Mar-Apr;68(2):448-453. doi: 10.4103/0028-3886.284374.

Abstract

BACKGROUND

Spinal hemangiopericytoma is very rare tumors with only a few case reports and one case series. We have treated ten patients between 2004 and 2017 and, thus, present a retrospective review of our patients with a focus on clinical presentation, radiological features, management, pathology, and outcome.

MATERIALS AND METHODS

Histopathological data were reviewed in all the cases and clinical and follow-up details were collected from data available in our department.

RESULTS

There were five males and five females, including one pediatric patient. The mean age of the patients was 34.7 years (Range 12-52 years). Dorsal, cervical, and lumbar spine involvement were found in five, four, and one patient, respectively. Intradural extramedullary tumor was the most common tumor and all patients presented motor weaknesses. Gross total resection of the tumor was done in seven patients and six patients received postoperative radiotherapy. Histopathology showed anaplastic tumor in two cases with high MIB-1 labelling index. Most patients were positive for CD34, vimentin, mic-2, and bcl-2. While the seven patients who underwent gross total resection improved significantly and were self-ambulatory in the follow-up period, two patients who underwent subtotal resection expired due to tumor metastasis.

CONCLUSION

Spinal hemangiopericytoma is a very rare tumor. We present a series of cases treated at our institute for the same. Gross total resection is the goal and radiotherapy should be given in case of residual tumor or high-grade tumors. Prognosis is good after gross total excision and functional recovery can be expected in most patients.

摘要

背景

脊髓血管外皮细胞瘤是一种非常罕见的肿瘤,仅有少数病例报告和一个病例系列。我们在 2004 年至 2017 年间治疗了 10 名患者,因此对我们的患者进行了回顾性分析,重点关注临床表现、影像学特征、治疗、病理学和预后。

材料和方法

对所有病例进行了组织病理学数据分析,并从我们科室的可用数据中收集了临床和随访细节。

结果

患者中男性 5 例,女性 5 例,包括 1 例儿科患者。患者的平均年龄为 34.7 岁(范围 12-52 岁)。5 例患者为脊柱背侧、颈椎和腰椎受累,4 例和 1 例患者分别为脊髓硬膜外肿瘤和脊髓髓内肿瘤。所有患者均表现为运动无力。7 例患者行肿瘤全切除,6 例患者术后行放疗。组织病理学显示 2 例为间变肿瘤,MIB-1 标记指数高。大多数患者 CD34、波形蛋白、mic-2 和 bcl-2 阳性。7 例行肿瘤全切除患者在随访期间明显改善,能够自行活动,2 例行次全切除患者因肿瘤转移死亡。

结论

脊髓血管外皮细胞瘤是一种非常罕见的肿瘤。我们报告了在我们研究所治疗的一系列病例。肿瘤全切除是目标,如果有肿瘤残留或高级别肿瘤,应给予放疗。肿瘤全切除后预后良好,大多数患者可恢复功能。

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