Doddamani Ramesh S, Meena Rajesh K, Selvam Murli M, Venkataramanaa Neelam K, Tophkhane Madhvi, Garg Sharat K
Department of Neurosurgery, All India Institute of Medical Sciences (AIIMS), New Delhi, India.
Department of Neurosurgery, Neurosurgical Education and Training School (NETS), All India Institute of Medical Sciences (AIIMS), New Delhi, India.
World Neurosurg. 2016 Jun;90:707.e5-707.e12. doi: 10.1016/j.wneu.2016.03.033. Epub 2016 Mar 19.
Gliosarcomas are rare, extremely high-grade, bimorphous malignant tumors of the central nervous system. Intraventricular location is extremely rare, and only a few case reports exist in the literature. The aim of our study is to review clinical, radiologic, and pathologic features of this unique oncological entity and report this rare case of primary cystic intraventricular gliosarcoma (IVGS) with a mural nodule.
A 23-year-old man had a 6-month history of headache and a single episode of generalized seizure. Examination revealed grade 1 papilledema. Brain magnetic resonance imaging revealed a cystic lesion with a mural nodule located within the occipital horn of the right lateral ventricle, which exhibited an intense enhancement of the nodule with patchy rim enhancement of the wall on gadolinium administration. The patient underwent right parietal craniotomy and gross total excision of the tumor.
Postoperative computed tomography of the brain showed evidence of complete tumor excision. The postoperative course of the patient was uneventful. Histopathologic analysis revealed malignant tumor comprising both glial and mesenchymal components suggestive of gliosarcoma.
Primary IVGS is an extremely rare malignancy, with only 9 cases reported in the literature, and it should be considered in the differential diagnosis of lateral ventricular tumors.
胶质肉瘤是中枢神经系统罕见的、高度恶性的双相性肿瘤。位于脑室内的情况极为罕见,文献中仅有少数病例报告。本研究的目的是回顾这种独特肿瘤实体的临床、放射学和病理学特征,并报告这例罕见的原发性脑室内囊性胶质肉瘤(IVGS)伴壁结节的病例。
一名23岁男性有6个月的头痛病史,并有一次全身性癫痫发作。检查发现1级视乳头水肿。脑部磁共振成像显示右侧脑室枕角内有一个带壁结节的囊性病变,钆剂注射后结节呈现明显强化,壁呈斑片状边缘强化。患者接受了右顶骨开颅手术并肿瘤全切。
术后脑部计算机断层扫描显示肿瘤已完全切除。患者术后过程顺利。组织病理学分析显示恶性肿瘤包含胶质和间充质成分,提示为胶质肉瘤。
原发性IVGS是一种极其罕见的恶性肿瘤,文献中仅报道了9例,在侧脑室肿瘤的鉴别诊断中应予以考虑。