Tamaki Masashi, Aoyagi Masaru, Kuroiwa Toshihiko, Yamamoto Masaaki, Kishimoto Seiji, Ohno Kikuo
Department of Neurosurgery, Graduate School, Tokyo Medical and Dental University, Yushima, Bunkyo-ku, Tokyo, Japan.
Skull Base. 2007 Sep;17(5):331-40. doi: 10.1055/s-2007-986438.
The management of clival chordoma remains problematic. We present the case of a 48-year-old woman with clival chordoma who underwent multiple surgeries and radiation therapy, including gamma knife stereotactic radiosurgery (GK-SRS), during a 10-year clinical course. The tumor was initially removed by gross total resection via the trans-sphenoidal approach, followed by external linac radiation therapy. The tumor recurred at the clivus 5 years after the initial operation. After repeated trans-sphenoidal removal of recurrent tumors, she twice underwent GK-SRS for a tumor remnant adjacent to the brainstem. Although this part of the tumor was controlled by GK-SRS, there was further tumor extension toward the sphenoid and maxillary sinuses. Ultimately, lower cranial nerve dysfunction developed due to tumor extension into the lower part of the clivus and the patient died of respiratory failure. Autopsy revealed the tumor to extend from the lower clivus to the bilateral middle fossae. The lower part of the tumor extended to the nasal cavity and to the posterior wall of the pharynx, resulting in compression of the upper pharyngeal region. The tumor around the jugular foramen compressed the lower cranial nerves bilaterally. Tumor cells did not, however, invade the intradural space microscopically. Although chordoma is not biologically malignant, this tumor can show massive extension with destruction of bony structures and extracranial invasion of connective tissues. Therefore, the optimal treatment strategy is to remove the tumor mass as extensively as possible, including normal bony structures and connective tissues surrounding the tumor, using skull base surgical techniques.
斜坡脊索瘤的治疗仍然存在问题。我们报告了一例48岁患有斜坡脊索瘤的女性病例，在10年的临床病程中，她接受了多次手术和放射治疗，包括伽玛刀立体定向放射外科治疗（GK-SRS）。肿瘤最初通过经蝶窦入路进行全切除，随后接受直线加速器外照射放疗。初始手术后5年肿瘤在斜坡复发。在反复经蝶窦切除复发性肿瘤后，她因脑干旁肿瘤残余两次接受GK-SRS治疗。尽管这部分肿瘤通过GK-SRS得到控制，但肿瘤进一步向蝶窦和上颌窦扩展。最终，由于肿瘤扩展至斜坡下部导致低位颅神经功能障碍，患者死于呼吸衰竭。尸检显示肿瘤从斜坡下部延伸至双侧中颅窝。肿瘤下部延伸至鼻腔和咽后壁，导致上咽部受压。颈静脉孔周围的肿瘤双侧压迫低位颅神经。然而，肿瘤细胞在显微镜下并未侵犯硬膜内间隙。尽管脊索瘤并非生物学上的恶性肿瘤，但该肿瘤可呈广泛扩展，破坏骨质结构并侵犯颅外结缔组织。因此，最佳治疗策略是尽可能广泛地切除肿瘤块，包括肿瘤周围的正常骨质结构和结缔组织，采用颅底外科技术。
