Pencovich Niv, Bot Gyang, Lidar Zvi, Korn Akiva, Wostrack Maria, Meyer Bernhard, Bydon Mohamad, Jallo George, Constantini Shlomi
Pediatric Neurosurgery, Dana-Dwek Children's Hospital, Tel-Aviv Sourasky Medical Center, Sackler Faculty of Medicine, Tel-Aviv University, 6 Weizmann Street, Tel Aviv, 64239, Israel.
Acta Neurochir (Wien). 2014 Jun;156(6):1215-22. doi: 10.1007/s00701-014-2048-2. Epub 2014 Mar 8.
Ependymomas are the most common glial neoplasms in the spinal cord. However, spinal cord ependymomas presenting with regional dissemination along the neuroaxis are rare, with a yet undetermined standard of care. We retrospectively evaluated the management and outcomes of patients who were diagnosed with spinal ependymoma with regional metastases at presentation (SERMP).
Between 2002 and 2012, 16 patients with regionally metastatic spinal ependymomas were diagnosed and treated. The patients were retrospectively divided into two groups according to tumor grading and histological features. Nine patients were diagnosed with myxopapillary ependymomas (MPE), and seven patients were diagnosed with other low-grade ependymomas.
With a median follow-up of 46.4 months, 13 out of 16 patients had no postsurgical recurrence/progression of the disease. In three patients, the disease recurred/progressed, leading to death in one patient. There was no correlation between gross total removal (GTR) of the main tumor, or resection of the main lesion and the metastatic foci and increased progression free survival in patients of the MPE group. There was an advantage for patients diagnosed with other low-grade ependymomas. Adjuvant radiotherapy did not prove beneficial.
SERMP has a relatively benign course. Achieving GTR of both the main lesion and the metastases is preferable, but should not be achieved at any cost, especially in MPE interfering with the conus medullaris. The benefit of adjuvant radiotherapy remains unproven.
室管膜瘤是脊髓中最常见的神经胶质瘤。然而，沿神经轴呈区域播散的脊髓室管膜瘤很少见，目前尚无确定的治疗标准。我们回顾性评估了初诊时被诊断为伴有区域转移的脊髓室管膜瘤（SERMP）患者的治疗及预后情况。
2002年至2012年期间，16例区域转移性脊髓室管膜瘤患者被诊断并接受治疗。根据肿瘤分级和组织学特征将患者回顾性分为两组。9例患者被诊断为黏液乳头型室管膜瘤（MPE），7例患者被诊断为其他低级别室管膜瘤。
中位随访时间为46.4个月，16例患者中有13例术后无疾病复发/进展。3例患者疾病复发/进展，其中1例死亡。MPE组患者的主要肿瘤全切除（GTR）、主要病变及转移灶切除与无进展生存期延长之间无相关性。诊断为其他低级别室管膜瘤的患者具有优势。辅助放疗未显示出益处。
SERMP病程相对良性。主病灶和转移灶均实现GTR是可取的，但不应不惜任何代价，尤其是对于累及脊髓圆锥的MPE。辅助放疗的益处仍未得到证实。
