Makhmudov U B, Mukhametzhanov D Zh, Usachev D Iu
Zh Vopr Neirokhir Im N N Burdenko. 1998 Apr-Jun(2):28-31.
Chordomas are relatively rare dysontogenetic tumors developing from the remnants of the spinal chord. Intracranial chordomas consist of 85% of all chordomas. Slowly progressive growth with destructive osteolysis in adjacent bones and low metastatic potential are specific for chordomas. Ninety six surgeries were performed in 71 patients with chordomas at the Burdenko Institute of Neurosurgery from 1987 to 1996. This article presents a clinical case of large clival chordoma with extra-intracranial growth in a 58 year-old female patient. The clinical picture is characterized by lesions in the caudal cranial nerves, predominantly on the right side, as well as cerebellar and brainstem signs and symptoms. Two-stage partial removal of malignant clival chordoma was performed via transoral and retrosigmoid approaches. The interval between the stages was 6 months. In the authors' opinion, multistage surgery is preferred in the treatment of giant clival chordomas. Partial removal should be started from the portions of a tumor producing the most prominent neurological signs. This method allows the patient's status to be temporarily improved.
脊索瘤是一种相对罕见的发育异常肿瘤，由脊索残余组织发展而来。颅内脊索瘤占所有脊索瘤的85%。脊索瘤的特点是生长缓慢，对相邻骨骼具有破坏性骨质溶解，转移潜能低。1987年至1996年期间，布尔坚科神经外科研究所对71例脊索瘤患者进行了96次手术。本文介绍了一名58岁女性患者患有巨大斜坡脊索瘤并向颅外生长的临床病例。临床表现以尾侧颅神经病变为主，主要在右侧，伴有小脑和脑干的体征和症状。通过经口和乙状窦后入路对恶性斜坡脊索瘤进行了两阶段部分切除。两阶段之间的间隔为6个月。作者认为，多阶段手术是治疗巨大斜坡脊索瘤的首选方法。部分切除应从产生最明显神经体征的肿瘤部分开始。这种方法可以使患者的病情暂时得到改善。
