Kim Su-Hyeong, Bak Koang-Hum, Kim Dong Won, Kang Tae-Hoon
Department of Neurosurgery, Hanyang University Medical Center, Seoul, Korea.
J Korean Neurosurg Soc. 2010 Nov;48(5):448-51. doi: 10.3340/jkns.2010.48.5.448. Epub 2010 Nov 30.
Primary central nervous system (CNS) sarcomas are exceedingly rare, and, to the best of our knowledge, there has not yet been a report of intramedullary sarcoma. Here, we report a primary intradural intramedullary sarcoma of the spinal cord in a four-year-old boy who presented with low back pain and a radiculopathy involving both lower extremities. The tumor showed significant enhancement on magnetic resonance (MR) images due to its extreme vascularity. Gross total tumor removal was performed with microelectrical pulse recording, and the patient also received adjuvant radiotherapy and chemotherapy. After the operation, the patient's sensory deficits were improved. Because CNS dissemination is common, entire neuraxis evaluation is essential, although there was no evidence of dissemination in this case. The prognosis of primary CNS sarcoma is poor due to infiltrative nature and early CNS dissemination is common, and the treatment of choice is radical surgical resection. Adjuvant therapy is also beneficial with radiotherapy and chemotherapy.
原发性中枢神经系统（CNS）肉瘤极为罕见，据我们所知，尚未有髓内肉瘤的报道。在此，我们报告一名4岁男孩，患有脊髓原发性硬脊膜内髓内肉瘤，表现为腰痛和累及双下肢的神经根病。由于肿瘤血管极其丰富，在磁共振（MR）图像上显示出显著强化。采用微电脉冲记录进行了肿瘤全切，患者还接受了辅助放疗和化疗。术后，患者的感觉障碍得到改善。由于CNS播散很常见，尽管该病例没有播散证据，但全神经轴评估至关重要。原发性CNS肉瘤预后较差，因其具有浸润性且早期CNS播散常见，治疗选择是根治性手术切除。辅助放疗和化疗也有益处。
