Rech Fabien, Rigau Valerie, Fabbro Michel, Kerr Christine, Gauchotte Guillaume, Taillandier Luc, Duffau Hugues
Department of Neurosurgery, CHU Nancy, Nancy, France.
Department of Neuropathology, CHU de Montpellier, Montpellier, France.
J Neurol Surg A Cent Eur Neurosurg. 2014 Nov;75(6):485-90. doi: 10.1055/s-0034-1372437. Epub 2014 Jun 27.
Secondary gliosarcomas are rare tumors, especially those arising from a World Health Organization (WHO) grade II glioma not irradiated. We report a case with subtotal resection for a WHO grade II oligoastrocytoma, without adjuvant treatment, whose metaplastic transformation into gliosarcoma suddenly occurred 4 years later with meningeal dissemination. We show a favorable outcome after therapeutic management of this rare entity.
A 46 year-old woman underwent surgery for a right premotor WHO grade II oligoastrocytoma discovered incidentally. Because of a subtotal resection with only 1 cc of residue, no complementary therapy was given, and the patient enjoyed a normal life for 4 years. In the meantime, the magnetic resonance images performed every 6 months showed a very low growth rate. Suddenly, the tumor switched toward a gliosarcoma profile with meningeal dissemination.
Reoperation, radiotherapy, and chemotherapy were performed, enabling a control of the disease with 15 months of follow-up (i.e., with radiologic shrinkage of the multiple lesions and preservation of quality of life).
A delayed sarcomatous transformation can acutely occur with a low proliferation index in a nonirradiated WHO grade II oligoastrocytoma. Furthermore, an aggressive therapeutic strategy can allow control of secondary gliosarcomas, even in cases of leptomeningeal spreading.
继发性胶质肉瘤是罕见肿瘤，尤其是那些起源于未经放疗的世界卫生组织（WHO）二级胶质瘤的肿瘤。我们报告一例对WHO二级少突星形细胞瘤进行次全切除且未行辅助治疗的病例，该肿瘤在4年后突然发生化生转变为胶质肉瘤并伴有脑膜播散。我们展示了对这种罕见疾病进行治疗管理后的良好结果。
一名46岁女性因偶然发现的右运动前区WHO二级少突星形细胞瘤接受手术。由于次全切除后仅残留1立方厘米，未给予辅助治疗，患者正常生活了4年。在此期间，每6个月进行的磁共振成像显示肿瘤生长速度非常缓慢。突然，肿瘤转变为胶质肉瘤形态并伴有脑膜播散。
进行了再次手术、放疗和化疗，在15个月的随访中实现了疾病控制（即多个病灶影像学缩小且生活质量得以保留）。
在未经放疗的WHO二级少突星形细胞瘤中，低增殖指数情况下可急性发生延迟性肉瘤样转变。此外，积极的治疗策略可控制继发性胶质肉瘤，即使在软脑膜播散的情况下。
