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原发性脑胶质肉瘤:放射学和组织病理学特征

Primary gliosarcoma of the brain: radiologic and histopathologic features.

作者信息

Romero-Rojas Alfredo E, Diaz-Perez Julio A, Ariza-Serrano Lina M, Amaro Deirdre, Lozano-Castillo Alfonso

机构信息

National Institute of Cancer; Bogotá, Colombia -

出版信息

Neuroradiol J. 2013 Dec;26(6):639-48. doi: 10.1177/197140091302600606. Epub 2013 Dec 18.

Abstract

Gliosarcoma is a rare central nervous system (CNS) neoplasm with biphasic glial and non-glial malignant components. Here we describe the radiologic and histopathologic features observed in five cases of primary gliosarcoma. The mean age at diagnosis in the studied patients was 54.2 years; these patients were predominantly males (male:female ratio = 4:1). At diagnosis all patients had several clinical deterioration. The most common symptoms of presentation were: headache (5/5 cases), seizures (4/5 cases) and hemiparesis (1/5 cases). All the tumors were large (mean major diameter= 4.12±1.64 cm) at diagnosis as evidenced in computer tomography (CT) scans and magnetic resonance images (MRIs), with preferential involvement of the temporal lobe and frequent associated deviation of the midline structures. Other common characteristics identified on CT scans and MRIs were partial contrast medium uptake with annular pattern (5/5 cases), peripheral edema (5/5 cases), and central calcification (3/5 cases). In additional a peak of dye uptake was observed (4/5 cases) on MRI spectrometry. In the histopathology, the glial component showed malignant astrocytes, with high Ki67 (>60%) and p53 positivity; the sarcomatous components displayed pleomorphic spindle cells similarly with p53 positivity and high Ki67 (75-90%) in all cases. Dedifferentiation to pleomorphic sarcoma (two cases), fibrosarcoma (one case), leiomyosarcoma (one case) and MPNST (one case) were documented. All patients received radiotherapy/chemotherapy and had a median overall survival of ten months. The study of radiologic and histopathologic features in primary gliosarcomas of the brain is a priority to achieve early diagnosis that can be translated to better outcomes. Here we describe the radiologic and histopathologic features observed in a group of gliosarcoma patients with variable histopathologic dedifferentiation.

摘要

胶质肉瘤是一种罕见的中枢神经系统(CNS)肿瘤,具有双相性胶质和非胶质恶性成分。在此,我们描述了5例原发性胶质肉瘤的放射学和组织病理学特征。研究患者的诊断时平均年龄为54.2岁;这些患者以男性为主(男:女比例 = 4:1)。诊断时所有患者均有几种临床病情恶化情况。最常见的症状表现为:头痛(5/5例)、癫痫发作(4/5例)和偏瘫(1/5例)。如计算机断层扫描(CT)和磁共振成像(MRI)所示,所有肿瘤在诊断时都很大(平均长径 = 4.12±1.64 cm),优先累及颞叶且常伴有中线结构偏移。CT扫描和MRI上识别出的其他常见特征包括环形模式的部分造影剂摄取(5/5例)、周围水肿(5/5例)和中央钙化(3/5例)。此外,在MRI光谱分析中观察到造影剂摄取峰值(4/5例)。在组织病理学方面,胶质成分显示恶性星形胶质细胞,Ki67高表达(>60%)且p53阳性;肉瘤成分显示多形性梭形细胞,所有病例中p53同样阳性且Ki67高表达(75 - 90%)。记录到向多形性肉瘤(2例)、纤维肉瘤(1例)、平滑肌肉瘤(1例)和恶性周围神经鞘膜瘤(1例)的去分化情况。所有患者均接受了放疗/化疗,中位总生存期为10个月。研究脑原发性胶质肉瘤的放射学和组织病理学特征对于实现早期诊断至关重要,这可以转化为更好的治疗结果。在此,我们描述了一组具有不同组织病理学去分化的胶质肉瘤患者中观察到的放射学和组织病理学特征。

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