Sharma Shivani, Sharma Anurag, Lobo Geover, Nayak Madhukar, Pradhan Dinesh, Bindal Swati, Gogoi Kamakhya, Katara Rahul, Kini Lata, Mohanty Sambit K
Departments of Pathology and Laboratory Medicine, CORE Diagnostics, Gurgaon, Haryana, India.
Department of Neurosurgery, Father Muller Medical College and Hospital, Mangalore, Karnataka, India.
Pathol Res Pract. 2017 Jul;213(7):868-871. doi: 10.1016/j.prp.2017.03.005. Epub 2017 Mar 23.
Dura-based intracranial neoplasms include a wide range of primary and metastatic tumors, varying in their clinical, radiologic, morphologic, and immunophenotypic characteristics. At this anatomic location, sarcomas are rare, however, they exhibit close morphologic resemblances to meningioma. Herein we describe the third case of primary synovial sarcoma of the parafalcine region in a50-years-old female, who presented with left-sided hemiplegia. The radiologic survey revealed a 5.5cm×5.8cm contrast enhancing dura-based mass at the right parafalcine region with meningeal enhancement and edema in the surrounding areas. Morphologic evaluation exhibited a high-grade spindle cell neoplasm, with focal hemangiopericytomatous pattern. The tumor cells were diffusely immunoreactive for CD99, Bcl2, TLE-1, and vimentin. The Ki-67 proliferation index was 40%. Pancytokeratin was focally positive. Epithelial membrane antigen, progesterone receptor, CD34, S-100, and glial fibrillary acidic protein were negative. Fluorescence in situ hybridization confirmed tumor specific translocation t(X;18)(p11.2;q11.2). Hence, final diagnosis of synovial sarcoma was rendered. Primary meningeal synovial sarcoma should be considered in the differential of aggressive and high-grade dura-based tumors in view of their relative chemosensitivity and future prospect of a molecular target-based therapy. The index case highlights the importance of an extensive pathologic analysis of high-grade mesenchymal lesions of the meninges to arrive at a definitive diagnosis and differentiate such tumors from other usual dura-based tumors, which has important therapeutic and prognostic implications.
硬脑膜颅内肿瘤包括多种原发性和转移性肿瘤，其临床、放射学、形态学和免疫表型特征各不相同。在这个解剖位置，肉瘤很少见，然而，它们在形态上与脑膜瘤相似。在此，我们描述了一名50岁女性，她患有左侧偏瘫，是第三例发生在大脑镰旁区域的原发性滑膜肉瘤。影像学检查显示，右侧大脑镰旁区域有一个5.5cm×5.8cm的强化硬脑膜肿块，伴有脑膜强化及周围区域水肿。形态学评估显示为高级别梭形细胞瘤，具有局灶性血管外皮细胞瘤样形态。肿瘤细胞对CD99、Bcl2、TLE-1和波形蛋白呈弥漫性免疫反应。Ki-67增殖指数为40%。全细胞角蛋白局灶阳性。上皮膜抗原、孕激素受体、CD34、S-100和胶质纤维酸性蛋白均为阴性。荧光原位杂交证实肿瘤具有特异性易位t(X;18)(p11.2;q11.2)。因此，最终诊断为滑膜肉瘤。鉴于原发性脑膜滑膜肉瘤相对的化学敏感性以及基于分子靶点治疗的未来前景，在鉴别侵袭性和高级别硬脑膜肿瘤时应考虑到该病。该病例强调了对脑膜高级别间质性病变进行广泛病理分析以得出明确诊断并将此类肿瘤与其他常见硬脑膜肿瘤区分开来的重要性，这具有重要的治疗和预后意义。
