Mahalingam Preethika, Smith Sam, Lopez Juanita, Sharma Rajaei K, Millard Thomas, Thway Khin, Fisher Cyril, Reardon David A, Jones Robin, Nicholson Andrew G, Cunningham David, Welsh Liam, Sharma Bhupinder
The Royal Marsden Hospital NHS Foundation Trust, London, UK.
Institute of Cancer Research, London and Sutton, UK.
Rare Tumors. 2023 Jan 19;15:20363613231152333. doi: 10.1177/20363613231152333. eCollection 2023.
Myxopapillary ependymoma (MPE) is a primary tumor of the central nervous system (CNS), characteristically an indolent malignancy involving the spinal conus medullaris, Filum terminale or cauda equina. We present a rare case of MPE, recurrent in the pelvic soft tissue with eventual pleural and intra-pulmonary metastasis. Refractory to repeated gross resection, adjuvant radiotherapy, platinum-based chemotherapy and temozolomide exploitation of mutant somatic BRCA1 status with the addition of a poly (ADP-ribose); polymerase inhibitor (PARPi) in a novel combination regimen with olaparib-temozolomide (OT) has achieved stable radiological disease after 10 cycles.
黏液乳头型室管膜瘤（MPE）是中枢神经系统（CNS）的原发性肿瘤，典型表现为一种累及脊髓圆锥、终丝或马尾的惰性恶性肿瘤。我们报告了一例罕见的MPE病例，该病例在盆腔软组织复发，最终发生胸膜和肺内转移。尽管进行了多次肉眼切除、辅助放疗、铂类化疗以及利用突变体体细胞BRCA1状态并联合聚（ADP - 核糖）聚合酶抑制剂（PARPi）与奥拉帕利 - 替莫唑胺（OT）组成的新型联合方案使用替莫唑胺，但均无效。经过10个周期的治疗后，该方案使影像学疾病达到稳定状态。
