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采用自体干细胞移植治疗原发性脊髓原始神经外胚层肿瘤:病例报告及文献复习

Primary intraspinal primitive neuroectodermal tumor treated with autologous stem cell transplantation: case report and review of the literature.

作者信息

Nutman Amir, Postovsky Sergey, Zaidman Irina, Elhasid Ronit, Vlodavsky Euvgeni, Kreiss Yitshak, Ben Arush Myriam Weyl

机构信息

Israel Defense Forces, Medical Corps, Haifa, Israel.

出版信息

Pediatr Hematol Oncol. 2007 Jan-Feb;24(1):53-61. doi: 10.1080/08880010601001396.

Abstract

The authors describe the case of a 19-year-old female patient with a primary primitive neuroectodermal tumor (PNET) of the thoraco-lumbar spinal cord, who presented with acute urinary retention and back pain for 2 months. Magnetic resonance imaging revealed an intradural extramedullary tumor, 6.5 cm long, in the region of the conus medullaris. Histological examination disclosed a small round cell tumor with immunohistochemical characteristics of a peripheral PNET. Metastatic workup showed no evidence of an intracranial tumor or metastases outside the neuroaxis. The patient received multidisciplinary treatment, including surgical excision, irradiation of the entire cranio-spinal axis, and high-dose chemotherapy with autologous stem cell rescue. Presently, 24 months after diagnosis, the patient remains in complete remission.

摘要

作者描述了一名19岁女性患者的病例,该患者患有胸腰段脊髓原发性原始神经外胚层肿瘤(PNET),出现急性尿潴留和背痛2个月。磁共振成像显示在脊髓圆锥区域有一个6.5厘米长的硬脊膜内髓外肿瘤。组织学检查发现一个小圆细胞肿瘤,具有外周PNET的免疫组化特征。转移灶检查未发现颅内肿瘤或神经轴外转移的证据。患者接受了多学科治疗,包括手术切除、全颅脊柱轴照射以及自体干细胞救援的大剂量化疗。目前,诊断后24个月,患者仍处于完全缓解状态。

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