Saito Yuki, Takemura Sunao, Sakurada Kaori, Sato Shinya, Kayama Takamasa
Department of Neurosurgery, Yamagata University School of Medicine, Japan.
No Shinkei Geka. 2010 May;38(5):441-8.
Intracranial extraskeletal mesenchymal chondrosarcoma is a very rare tumor that affects young adults. We report a 31-year-old woman with a huge intracranial extraskeletal mesenchymal chondrosarcoma. She presented with a history of right homonymous hemianopsia for 6 months. Computed tomography scans demonstrated a heterogeneously enhanced mass, in the right occipital region, which was lobulated and contained areas of calcification. Magnetic resonance imaging demonstrated a large mass (67 mm) of heterogenous intensity that showed enhancement and was attached to the falx cerebri. The patient underwent right occipital craniotomy and subtotal removal of the tumor. At operation, the tumor was found to arise in the falx cerebri and the cranial vault was not involved. Pathologic examination revealed a mesenchymal chondrosarcoma. She underwent conventional radiotherapy after surgery and remained free of progression at the 20-month follow up. We review previously reported cases of extraskeletal intracranial mesenchymal chondrosarcoma and discuss imaging diagnosis, treatment strategies, and outcome.
颅内骨外间叶性软骨肉瘤是一种非常罕见的肿瘤,好发于年轻人。我们报告了一名31岁患有巨大颅内骨外间叶性软骨肉瘤的女性。她有6个月的右侧同向性偏盲病史。计算机断层扫描显示右侧枕叶区域有一个不均匀强化的肿块,呈分叶状,并有钙化区域。磁共振成像显示一个大肿块(67毫米),信号强度不均匀,有强化,附着于大脑镰。患者接受了右侧枕下开颅手术,肿瘤次全切除。手术中发现肿瘤起源于大脑镰,颅顶未受累。病理检查显示为间叶性软骨肉瘤。她术后接受了常规放疗,在20个月的随访中无病情进展。我们回顾了先前报道的颅内骨外间叶性软骨肉瘤病例,并讨论了影像学诊断、治疗策略及预后。