Recurrent Central Nervous System Medulloepithelioma: Response and Outcome Following Marrow-Ablative Chemotherapy With Stem Cell Rescue
Recurrent Central Nervous System Medulloepithelioma: Response and Outcome Following Marrow-Ablative Chemotherapy With Stem Cell Rescue
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复发性中枢神经系统髓上皮瘤：骨髓消融化疗联合干细胞救援后的反应和结果
DOI：
10.1097/01.mph.0000165498.24989.9a
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发表时间：
2005
期刊：
影响因子：
--
通讯作者：
J. Finlay
中科院分区：
文献类型：
--
作者：
Lindsey S Norris;S. Snodgrass;Douglas C. Miller;J. Wisoff;J. Garvin;L. Rorke;J. Finlay
髓上皮瘤是一种罕见的中枢神经系统原始神经外胚层肿瘤，通常在儿童时期发展，表现出高度恶性的行为，早期进展或复发。据报道，一旦复发，死亡就会以不变的速度紧随其后。本研究的目的是评价大剂量骨髓切除化疗联合自体造血干细胞挽救治疗复发性中枢神经系统髓上皮瘤的疗效。3名患有复发性中枢神经系统髓上皮瘤的儿童接受了大剂量的硫替巴和依托泊苷去髓化疗(1例)或加用卡铂(2例)。随后只有一名患者接受了放射治疗。1例治疗时有残留放射学肿瘤的儿童在接受骨髓切除化疗后，可评估疗效，并显示软脑膜疾病完全消失。两名儿童在接受骨髓切除化疗后2.0个月和5.5个月出现肿瘤复发。第三个孩子在接受治疗后12年内继续没有肿瘤。作者在骨髓切除化疗和自体造血干细胞挽救方面的经验表明，这种治疗策略可能有益于儿童髓上皮瘤的初始治疗方法。
Medulloepithelioma is a rare primitive neuroectodermal tumor of the central nervous system usually developing in childhood, displaying highly malignant behavior, with early progression or recurrence. Once a recurrence develops, death reportedly follows with invariable rapidity. The purpose of this study was to evaluate the efficacy of high-dose, marrow-ablative chemotherapy with autologous hemopoietic stem cell rescue in the treatment of recurrent central nervous system medulloepithelioma. Three young children with recurrent central nervous system medulloepithelioma received high-dose marrow-ablative chemotherapy with thiotepa and etoposide either alone (one patient) or with the addition of carboplatin (two patients). This was followed by irradiation only in one patient. One child with residual radiographic tumor at the time of treatment could be evaluated for response and showed complete resolution of leptomeningeal disease after receiving marrow-ablative chemotherapy. Two children developed tumor recurrence at 2.0 and 5.5 months after receiving marrow-ablative chemotherapy. The third child continues free of tumor beyond 12 years from treatment. The authors' experience with marrow-ablative chemotherapy and autologous hemopoietic stem cell rescue suggests that this treatment strategy might be beneficially incorporated into the initial treatment approach for young children with medulloepithelioma.