Kawabata Yasuhiro, Aoki Tomokazu, Yamamoto Tetsurou, Yasui Hisateru, Sawai Satoru, Fukuda Shunichi, Kawarazaki Satoru, Tsukahara Tetsuya
Department of Neurosurgery, National Hospital Organization Kyoto Medical Center, Kyoto, Kyoto, Japan.
Department of Pathology, National Hospital Organization Kyoto Medical Center, Kyoto, Kyoto, Japan.
NMC Case Rep J. 2017 Dec 7;5(1):1-7. doi: 10.2176/nmccrj.cr.2017-0048. eCollection 2018 Jan.
Primary intracranial leiomyosarcoma (LMS) is an extremely rare tumor of the central nervous system. Only sporadic case reports have been published, and therefore data regarding long-term prognosis remain scarce. A 76-year-old woman presented with a right parietal mass, which had grown rapidly in the month prior to admission. Neuroimaging showed a resemblance to intraosseous meningioma. Gross total resection of the tumor was achieved, and histological diagnosis confirmed LMS. Because positron emission tomography (PET) with fluorodeoxyglucose (FDG) just after the resection showed no abnormal uptake, we diagnosed the tumor as primary intracranial LMS. Follow-up PET at 16 months after treatment showed two foci of FDG uptake in the bilateral lungs. Histological diagnosis by surgical resection identified the lesions as lung metastases of LMS. In addition, follow-up head magnetic resonance imaging (MRI) at 31 months showed local recurrence, and we conducted salvage therapy using CyberKnife system (Accuray incorporated) and pazopanib. To date, for 15 months after local recurrence, she is alive with intracranial recurrent disease remained inactive.
原发性颅内平滑肌肉瘤（LMS）是一种极其罕见的中枢神经系统肿瘤。仅有散在的病例报告发表，因此关于长期预后的数据仍然匮乏。一名76岁女性因右顶叶肿块就诊，该肿块在入院前一个月迅速增大。神经影像学检查显示其与骨内脑膜瘤相似。肿瘤实现了全切除，组织学诊断证实为LMS。由于切除术后即刻进行的氟脱氧葡萄糖（FDG）正电子发射断层扫描（PET）未显示异常摄取，我们将该肿瘤诊断为原发性颅内LMS。治疗后16个月的随访PET显示双侧肺部有两个FDG摄取灶。手术切除的组织学诊断确定这些病变为LMS的肺转移。此外，31个月时的随访头部磁共振成像（MRI）显示局部复发，我们使用射波刀系统（Accuray公司）和帕唑帕尼进行了挽救治疗。迄今为止，在局部复发后的15个月里，她存活，颅内复发病灶未再活动。
