Aydin Ahmet Levent, Sasani Mehdi, Oktenoglu Tunc, Solaroglu Ihsan, Ozer Ali Fahir
Istanbul Physical Therapy and Rehabilitation Hospital, Department of Neurosurgery, Istanbul, Turkey.
Turk Neurosurg. 2013;23(4):551-6. doi: 10.5137/1019-5149.JTN.5666-11.2.
Chordoma is a rare, slow-growing primary malignant tumor of the axial skeleton, arising from the embryonic cells of primitive notochord. Chordomas may arise at different sites of the vertebral column simultaneously or more probably they may metastasise along the neural axis insidiously. Recurrence despite radical surgery and following adjuvant therapy is possible.
A 46-year-old female patient presented weakness and numbness of the lower extremities. She was operated for clivus chordoma five years ago at another institute.
First the patient underwent surgery for resection of the tumor at the cervical region. a second surgery was performed to resect tumor on the foramen magnum and at the C1 level. Histologic examination of the removed vertebra confirmed the diagnosis of chordoma involving the vertebral body. Radiotherapy was administered after the second surgery. Follow-up neurological and radiological examinations revealed no abnormal neurological symptoms 2,5 years after second surgery. There were no distant organ metastases.
A patient with diagnosed chordoma of the spine must be investigated with MRI of other regions of the neuraxis to exclude second or even third source of chordoma metastases. In metastatic chordoma cases, radical or gross total resection should be performed for each lesion but if complete surgical resections are impossible, preoperative or postoperative radiation therapy should be planned to improve life expectancy.
脊索瘤是一种罕见的、生长缓慢的原发性轴骨骼恶性肿瘤,起源于原始脊索的胚胎细胞。脊索瘤可同时发生于脊柱的不同部位,或者更有可能沿神经轴隐匿性转移。尽管进行了根治性手术及辅助治疗,仍有可能复发。
一名46岁女性患者出现下肢无力和麻木。她五年前在另一家机构接受了斜坡脊索瘤手术。
患者首先接受了颈椎区域肿瘤切除术。随后进行了第二次手术,切除枕骨大孔和C1水平的肿瘤。对切除的椎体进行组织学检查,确诊为累及椎体的脊索瘤。第二次手术后进行了放疗。第二次手术后2.5年的随访神经学和影像学检查显示无异常神经症状。无远处器官转移。
对于诊断为脊柱脊索瘤的患者,必须对神经轴的其他区域进行MRI检查,以排除脊索瘤转移的第二甚至第三来源。在转移性脊索瘤病例中,应对每个病灶进行根治性或大体全切,但如果无法完全手术切除,则应计划术前或术后放疗以提高预期寿命。