Gallagher Shannon J, Rosenberg Stephen A, Francis David, Salamat Shahriar, Howard Steven P, Kimple Randall J
Department of Human Oncology, University of Wisconsin School of Medicine and Public Health, United States.
Department of Pathology and Laboratory Medicine, University of Wisconsin School of Medicine and Public Health, United States.
Clin Neurol Neurosurg. 2018 Feb;165:76-80. doi: 10.1016/j.clineuro.2017.12.014. Epub 2018 Jan 6.
Primary leiomyosarcoma is a rare tumor in the CNS, with few reported cases. Here, we describe a case of a primary intracranial leiomyosarcoma of the tentorium cerebelli. A 43-year-old woman presented with headache, acute vision loss, and difficulty speaking. MRI revealed a large heterogeneous-enhancing occipital mass, which was subsequently resected and diagnosed as a primary intracranial leiomyosarcoma. The patient went onto adjuvant radiotherapy delivering 60 Gy in 30 fractions. These tumors are exceedingly rare in immunocompetent individuals. We reviewed the 16 cases that have been reported in the literature. Surgical resection was the most common treatment (92%) with 53% receiving adjuvant radiation. There currently is no standard treatment regimen for intracranial leiomyosarcomas. Additional case reports that include descriptive treatment approaches with patient outcomes may help ascertain the best approach to treating these malignancies.
原发性平滑肌肉瘤是中枢神经系统中一种罕见的肿瘤，报道的病例很少。在此，我们描述一例小脑幕原发性颅内平滑肌肉瘤病例。一名43岁女性出现头痛、急性视力丧失和言语困难。磁共振成像（MRI）显示枕部有一个大的不均匀强化肿块，随后切除并诊断为原发性颅内平滑肌肉瘤。患者接受了辅助放疗，分30次给予60 Gy剂量。这些肿瘤在免疫功能正常的个体中极为罕见。我们回顾了文献中报道的16例病例。手术切除是最常见的治疗方法（92%），53%的患者接受了辅助放疗。目前颅内平滑肌肉瘤尚无标准治疗方案。更多包括描述性治疗方法及患者预后的病例报告可能有助于确定治疗这些恶性肿瘤的最佳方法。
