Nutman Amir, Postovsky Sergey, Zaidman Irina, Elhasid Ronit, Vlodavsky Euvgeni, Kreiss Yitshak, Ben Arush Myriam Weyl
Israel Defense Forces, Medical Corps, Haifa, Israel.
Pediatr Hematol Oncol. 2007 Jan-Feb;24(1):53-61. doi: 10.1080/08880010601001396.
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.
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。