Ye Wei-Biao, Zhou Jian-Ping, Xu Yong-Qiang, Lu Bi-Yan, Li Zhong-Jun
Department of Thoracic Surgery and Pathology, Dongguan People's Hospital of Southern Medical University.
Department of Basic Medical Sciences, Dongguan Polytechnic, Dongguan, Guangdong Province, China.
Medicine (Baltimore). 2019 Nov;98(44):e17686. doi: 10.1097/MD.0000000000017686.
Ependymomas are neuroepithelial tumors that typically occur in the central nervous system. Ependymomas arising in the mediastinum are exceedingly rare, with only approximately 9 isolated cases reported in the literature to date.
A 35-year-old woman was referred to our hospital with complaints of progressive back pain for 3 months. Physical examination revealed decreased breathing sounds and tenderness. Contrast-enhanced computed tomography showed a soft tissue mass with heterogeneous enhancement in the right posterior mediastinum.
The diagnosis of primary mediastinal ependymomas (PMEs) was confirmed by postoperative histopathologic examination.
The patient underwent surgical resection of the tumor and experienced local recurrence with neck metastasis 2 years postoperatively. She underwent reoperation for the recurrent tumors and received postoperative radiotherapy and adjuvant chemotherapy. Two years later, the patient is doing well, with no evidence of tumor progression or recurrence.
Since PMEs are exceedingly rare, treatment options are limited. Surgical resection seems to be the mainstay of treatment. Further evidence-based studies are required to prove the benefit of radiotherapy and chemotherapy in the treatment of PMEs.
室管膜瘤是一种神经上皮肿瘤,通常发生于中枢神经系统。纵隔内发生的室管膜瘤极为罕见,迄今为止文献中仅报道了约9例孤立病例。
一名35岁女性因进行性背痛3个月转诊至我院。体格检查发现呼吸音减弱及压痛。增强计算机断层扫描显示右后纵隔有一软组织肿块,强化不均匀。
术后组织病理学检查确诊为原发性纵隔室管膜瘤(PME)。
患者接受了肿瘤手术切除,术后2年出现局部复发并伴有颈部转移。她接受了复发性肿瘤的再次手术,并接受了术后放疗和辅助化疗。两年后,患者情况良好,无肿瘤进展或复发迹象。
由于PME极为罕见,治疗选择有限。手术切除似乎是主要的治疗方法。需要进一步的循证研究来证明放疗和化疗在PME治疗中的益处。