椎管内恶性孤立性纤维性肿瘤1例及文献复习

Malignant solitary fibrous tumor of spinal canal : report of a case and review of literatures

目的:探讨椎管恶性孤立性纤维性肿瘤的临床病理特征、鉴别诊断及治疗和预后。方法:报道1例椎管内恶性孤立性纤维性肿瘤的临床病理资料并复习文献。结果:患者女性,80岁。因"反复颈部疼痛1年,加重伴四肢麻木乏力2个月余"入院。CT示颈3至颈5平面椎管内髓外硬膜下3.0 cm×1.5 cm×1.0 cm占位。结果考虑为脊膜瘤行手术。镜检可见肿瘤由短梭形或卵圆形细胞组成,排列成束状、漩涡状,富于胶原纤维,部分区呈血管外皮瘤样结构,瘤细胞有异型性,核分裂象多见(≥4个/10 HPF)。免疫表型检查示,肿瘤细胞波形蛋白、CD34弥漫阳性,CD99、bcl-2阳性。病理诊断,颈3至颈5椎管内低度恶性孤立性纤维性肿瘤。结论:椎管内恶性孤立性纤维性肿瘤非常罕见,确诊主要依靠病理形态学及免疫组织化学标记,并应与椎管内其他梭形细胞肿瘤进行鉴别。

Objective To study the clinical pathological features,immunohistochemical characteristics and differential diagnosis of solitary fibrous tumor（SFT） of spinal canal.Methods One case of malignant SFT of spinal canal was reported and relevant literatures were reviewed.Results The patient was a 80 years old woman with neckache for a year and aggravating for two months with numbness and weakness of four limbs.CT showed an intradural,extramedullary mass from C3 to C5 with a size of 3.0 cm×1.5 cm×1.0 cm.The patient was operated with a presumed diagnosis of intra-spinal meningioma.Histopathologically,the tumor was composed of spindle-shaped cells with fascicular,storiform arrangement and plenty of collagen and showing a haemangiopericytoma-like structure.The cells were pleomorphic with mitotic count ≥4 per 10 high power fields.Immunohistochemically,the tumor cells were diffusely positive for vimentin,CD34 and positive for CD99 and bcl-2.The pathological diagnosis was low grade malignant SFT of C3 to C5 cervical canal.Conclusions Malignant SFT of spinal canal is a rare tumor.Diagnosis is based on histopathological and immunohistochemical findings.Differential diagnosis includes other spindle-shaped cell tumors of spinal canal.