Chopra Ruchi, Bhardwaj Minakshi, Premsagar Ishwar Chand
Department of Pathology, PGIMER & Dr RMLH, New Delhi, India.
Rare Tumors. 2010 Mar 31;2(1):e3. doi: 10.4081/rt.2010.e3.
Meningeal fibrosarcomas are rare tumors. Only 41 cases have been reported in the literature to date. Primary central nervous system fibrosarcomas are very aggressive neoplasms and have a poor prognosis. Hence they need to be correctly diagnosed. This is a case of a 13-year old boy with intracranial space occupying lesion. The mass was completely removed and histological examination was characteristic of meningeal fibrosarcoma. The pathological diagnosis is usually made on routine light microscopic examination; however, occasionally these may be difficult to distinguish from other malignant neoplasms such as gliomas, meningiomas and metastases. The diagnosis of fibrosarcoma is based on the identification of a predominant herringbone architectural pattern, the overall uniformity of the spindle cell population, the prominent vimentin positivity, and the presence of pericellular reticulin fibre network. IHC helps to exclude other diagnoses.
脑膜纤维肉瘤是罕见肿瘤。迄今为止,文献中仅报道过41例。原发性中枢神经系统纤维肉瘤是极具侵袭性的肿瘤,预后较差。因此,需要对其进行正确诊断。这是一例13岁男孩患有颅内占位性病变的病例。肿块被完全切除,组织学检查具有脑膜纤维肉瘤的特征。病理诊断通常通过常规光学显微镜检查做出;然而,偶尔这些肿瘤可能难以与其他恶性肿瘤如胶质瘤、脑膜瘤和转移瘤区分开来。纤维肉瘤的诊断基于识别主要的人字形结构模式、梭形细胞群体的整体一致性、明显的波形蛋白阳性以及细胞周围网状纤维网络的存在。免疫组化有助于排除其他诊断。