Riegel Devon C, Fonkem Ekokobe, Connelly Jennifer M
Neurology, Medical College of Wisconsin, Milwaukee, USA.
Cureus. 2024 Aug 27;16(8):e67928. doi: 10.7759/cureus.67928. eCollection 2024 Aug.
Myxopapillary ependymomas (MPEs) are rare tumors of the central nervous system, and outcomes are generally worse with recurrent disease. These tumors can rarely metastasize outside the neuraxis. We present a case of a 35-year-old female with a history of MPEs who developed extraneural metastases 11 years after her initial gross total resection. Sites of metastases included multiple bilateral intrapulmonary and pleural-based masses with pleural effusion and a pelvic mass. The patient was treated with dose-dense TMZ and lapatinib and had a mixed radiographic response after 12 cycles of treatment. This is the first known case of extraneural metastases of MPEs to demonstrate a radiographic response to dose-dense TMZ and lapatinib. This case presentation discusses the need to establish optimal treatment of extraneural ependymal metastases, duration of treatment, and strategy for the management of recurrent diseases.
黏液乳头型室管膜瘤（MPEs）是中枢神经系统的罕见肿瘤，复发疾病的预后通常更差。这些肿瘤很少会转移至神经轴以外。我们报告一例35岁女性黏液乳头型室管膜瘤患者，在初次肿瘤全切术后11年发生了神经外转移。转移部位包括双侧多个肺内及胸膜下肿块伴胸腔积液以及一个盆腔肿块。该患者接受了剂量密集型替莫唑胺（TMZ）和拉帕替尼治疗，治疗12个周期后影像学表现为混合反应。这是已知首例黏液乳头型室管膜瘤神经外转移对剂量密集型替莫唑胺和拉帕替尼呈现影像学反应的病例。本病例报告讨论了确立神经外室管膜瘤转移的最佳治疗方法、治疗持续时间以及复发性疾病管理策略的必要性。
