伴骶骨广泛破坏的黏液乳头型室管膜瘤1例临床病理分析

Myxopapillary ependymoma with extensive sacral destruction: a clinicopathologic analysis

目的探讨伴骶骨广泛破坏的黏液乳头型室管膜瘤的临床病理学特点、诊断及鉴别诊断。方法对1例骶骨广泛性破坏黏液乳头型室管膜瘤的临床和影像学资料、病理学特征及免疫表型进行观察分析。结果患者,女性,73岁,腰骶部疼痛不适1个月,不慎摔伤腰骶部入院,骶尾椎MRI示骶2-3椎体肿瘤,考虑恶性。组织学形态示肿瘤细胞呈立方或长梭形放射状排列在血管间质轴心周围,形成乳头状结构,乳头缺乏的区域,常见黏液形成微囊,并将肿瘤细胞和玻璃样变的血管轴心隔开,肿瘤细胞无多形性,核分裂像少见。免疫组化结果:GFAP(+),S100(+),Vimentin(+),EMA(-),CKpan(-),Ki67(<1%阳性)。结论伴骶骨广泛溶骨性破坏的黏液乳头型室管膜瘤少见,具有惰性生物学行为,生长缓慢,切除不净可复发,特别需要与发生在骶骨的脊索瘤和转移性癌相鉴别。

Objective To investigate the clinical and pathological features,diagnosis,and differential diagnosis of myxopapillary ependymoma ( MPE) with extensive sacral destruction. Methods Retrospective analysis of the clinical and radiological data,histopathological morphology,and immunophenotype was conducted for one case of MPE with extensive sacral destruction. Results The patient,female,73 years of age,with a history of low backache for one month,was admitted to hospital with lumbosacral injury. MRI image demonstrated lesions involving vertebral bodies of S2-3. Microscopically,the lesion was composed of cuboidal to elongated tumour cells radially arranged in a papillary manner around vascularied stromal cores. In areas without nipples,myxoid matrix material accumulated between tumour cells and hyaline blood vessels. The tumour cells showed no pleomorphism, with low mitotic activity. Immunohistochemically,tumour cells were positive for GFAP,S100,and Vimentin,and negative for EMA and cytokeratin. Ki67 labeling index was < 1% positive. Conclusion MPE with extensive sacral destruction is a rare tumor with indolent behaviour. Late recurrence may occur with incomplete resection. Differential diagnosis include chordoma and metastatic carcinoma.