Chondrosarcoma of the skull base

Chondrosarcoma of the skull base
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DOI:
10.1007/s11060-005-5981-3
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发表时间:
2006-01-01
影响因子:
3.9
通讯作者:
Liebsch, N
Liebsch, N
中科院分区:
医学2区
文献类型:
--
作者:
Baehring, JM;Piepmeier, J;Liebsch, N

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一名15岁男孩主诉枕部头痛在两周内恶化。他感到头晕,身体不稳。在神经学检查中,他的舌头右侧有纤颤,并向右偏移。右侧有凝视诱发的水平眼震。磁共振成像显示一破坏性病变累及枕骨和右岩骨[C: CT, D: t1加权MRI加钆]。脑血管造影显示右侧椎动脉在C1椎体弓水平与肿瘤相邻处出现不规则且局灶性狭窄段[E]。经枕下、乳突和颞部入路行次全切除。肿瘤完全在硬膜外包裹脑神经IX, X, XI和XII。这些都是自由解剖的。组织病理学检查显示肿瘤细胞排列成大小形状不规则的小叶。小叶由软骨基质组成,由纤维组织的薄带分开。肿瘤细胞(插图)细胞核增厚,深染;有丝分裂象很少遇到(箭头)[A];白条= 100微米。黑条(插入)= 25微米。苏木精和伊红染色]。肿瘤的其他区域呈多细胞性,显示局灶性黏液样变,伴罕见的星状细胞(插图,箭头)[B];黑条= 50微米。苏木精和伊红染色]。诊断为软骨肉瘤,II级。对残余肿瘤行立体定向质子束放射治疗。
A 15 year old boy complained of an occipital headache worsening over the course of two weeks. He felt ‘dizzy’and unsteady. On neurological examination, fibrillations were seen on the right side of his tongue which deviated to the right. There was gaze-evoked horizontal nystagmus to the right. Magnetic resonance imaging revealed a destructive lesion involving the occipital and right petrous bone [C: CT, D: T1-weighted MRI with gadolinium]. A cerebral angiogram showed an irregular and focally narrowed segment of the right vertebral artery adjacent to the tumor at the level of the arch of the C1 vertebral body [E]. A subtotal resection via a suboccipital, transmastoid and transtemporal approach was performed. The tumor was entirely extradural encasing cranial nerves IX, X, XI and XII. These were dissected free. Histopathological examination demonstrated tumor cells arranged in lobules of irregular size and shape. The lobules consisted of a chondroid matrix and were separated by thin bands of fibrous tissue. The tumor cells (inset) showed enlarged hyperchromatic nuclei; mitotic figures were rarely encountered (arrow)[A; white bar= 100 micron. Black bar (inset)= 25 micron. Hematoxylin and eosin stain]. Other areas of the tumor were hypercellular and displayed focal myxoid changes with rare stellate cells (inset, arrow)[B; black bars= 50 micron. Hematoxylin and eosin stain]. Findings were diagnostic for chondrosarcoma, grade II. For the residual tumor, stereotactic proton beam radiation therapy was provided.