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颅内恶性孤立性纤维瘤。

An intracranial malignant solitary fibrous tumor.

机构信息

Department of Neurosurgery, Ilsan Baek Hospital, College of Medicine, Inje University, Goyang-Si, Gyeonggi-Do, Republic of Korea.

出版信息

Neuropathology. 2011 Apr;31(2):177-82. doi: 10.1111/j.1440-1789.2010.01148.x.

Abstract

Intracranial malignant solitary fibrous tumor (SFT) is very rare. It was identified in a 39-year-old female patient who underwent malignant transformation over 6 months. MRI revealed an 8 × 5 × 6 cm mass with heterogenous strong enhancement in the left occipital lobe. Histologic findings and immunophenotype (positive for CD34, bcl-2 and vimentin, and negative for epithelial membrane antigen or S100 protein) of the primary tumor were typical of SFT. However, there was a focal area (<10% of tumor volume) showing hypercellularity, nuclear pleomorphism and increased Ki-67 labeling index (LI) of 10% (average, 1%). At the second operation, the recurrent tumor revealed full-blown histologic features of malignant SFT, such as infiltrative brain invasion, marked nuclear pleomorphism, frequent mitotic figures (15/10 high power fields), and high Ki-67 LI (25%). The presence of atypical histologic finding or increased Ki-67 LI in the typical SFT, although it is focal, needs to be mentioned in the diagnosis and also may require more aggressive surgical management.

摘要

颅内恶性孤立性纤维瘤(SFT)非常罕见。一位 39 岁女性患者在 6 个月内发生恶性转化,被确诊为颅内恶性孤立性纤维瘤。MRI 显示左枕叶有一个 8×5×6cm 的肿块,呈异质性明显强化。原发性肿瘤的组织学表现和免疫表型(CD34、bcl-2 和波形蛋白阳性,上皮膜抗原或 S100 蛋白阴性)典型为 SFT。然而,有一个局灶区域(<肿瘤体积的 10%)显示细胞密度增加、核异型性和 Ki-67 标记指数(LI)升高(10%,平均为 1%)。在第二次手术中,复发性肿瘤显示出恶性 SFT 的全面组织学特征,如脑浸润、明显的核异型性、频繁的有丝分裂(10 个高倍视野中有 15 个)和高 Ki-67 LI(25%)。在典型的 SFT 中存在非典型的组织学发现或 Ki-67 LI 升高,尽管是局灶性的,但在诊断中需要提及,也可能需要更积极的手术治疗。

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