Liu Xiaohai, Li Mingchu, Chen Ge
Department of Neurosurgery, Xuanwu Hospital Capital Medical University, Beijing, China.
Transl Cancer Res. 2022 Sep;11(9):3426-3433. doi: 10.21037/tcr-22-211.
Clival chordoma is a locally aggressive tumor originating from remnants of the embryologic notochord. Although clival chordomas account for only 0.2% of all central nervous system tumors, they are characterized by local invasion and destruction, dural invasion, bone erosion, and cranial nerve palsy, and even metastasis.
We report a case of a 49-year-old female with an intradural spinal seeding metastasis 16 months after the initial endoscopic endonasal surgery (EES) for a clival chordoma. Gross total resection of tumor in upper clival region was achieved after initial EES and pathology revealed a classic chordoma. After 10 months, follow-up magnetic resonance (MR) showed a recurrence and gamma knife was applied. After 16 months, the patient complained of neck pain and MR showed a new lesion in the spinal canal at C1 to C2 level. After craniotomy, the lesion in the spinal canal was totally removed, and pathology confirmed a chordoma with increased proliferative potential. The spinal chordoma might have occurred as a result of intradural spinal seeding metastasis through cerebral spinal fluid the during the initial operation.
Chordomas are not only locally aggressive but also unpredictable and may metastasize through cerebral spinal fluid. Intensive follow-up is of great importance in the long term postoperatively time for clival chordoma patients.
斜坡脊索瘤是一种起源于胚胎脊索残余组织的局部侵袭性肿瘤。尽管斜坡脊索瘤仅占所有中枢神经系统肿瘤的0.2%，但其特点是局部侵袭和破坏、硬膜侵袭、骨质侵蚀和颅神经麻痹，甚至发生转移。
我们报告一例49岁女性患者，在初次经鼻内镜手术（EES）切除斜坡脊索瘤16个月后出现硬脊膜内脊髓播散转移。初次EES后实现了斜坡上部区域肿瘤的全切除，病理显示为典型的脊索瘤。10个月后，随访磁共振成像（MR）显示复发，遂行伽玛刀治疗。16个月后，患者主诉颈部疼痛，MR显示C1至C2水平椎管内出现新病变。开颅术后，椎管内病变被完全切除，病理证实为具有较高增殖潜能的脊索瘤。脊髓脊索瘤可能是由于初次手术期间通过脑脊液发生硬脊膜内脊髓播散转移所致。
脊索瘤不仅具有局部侵袭性，而且不可预测，可能通过脑脊液转移。对于斜坡脊索瘤患者，术后长期进行密切随访非常重要。
