摘要：

研究背景 由于斜坡位置的特殊性（被诸多解剖结构阻挡），加之斜坡恶性肿瘤病理类型复杂多样，使其手术难度和风险明显增加。为了提高诊断准确性和手术安全性，从而有效改善患者预后，拟对斜坡恶性肿瘤患者手术入路适应证，以及个体化、规范化的综合治疗方案进行回顾总结，以为临床提供参考。方法与结果 2007年7月至2018年12月共确诊56例斜坡恶性肿瘤患者，临床表现以顽固性头痛和渐进性脑神经麻痹为主。经多学科综合评估，共45例行肿瘤根治性切除术，分别经鼻（15例占33.33%）、经口腭联合口鼻（7例占15.56%）、Derome（8例占17.78%）、上颌骨掀翻（9例占20%）和经颞下（6例占13.33%）入路；11例行组织活检术；病理类型以脊索瘤（28例占50%）为主；达全切除者占46.67%（21/45例）、次全切除37.78%（17/45例）、部分切除15.56%（7/45例）；共28例术后发生并发症，包括神经功能障碍（13例次占23.21%）、脑脊液鼻漏（14例次占25%）、颅内感染（8例次占14.29%）、肺部感染（2例次占3.57%）、术区出血（2例次占3.57%）、精神症状（1例次占1.79%）等；围手术期分别接受适形调强、立体定向放射治疗或同步放化疗。平均随访59.90个月，其中8例（14.29%）失访、16例（28.57%）疾病无进展、13例（23.21%）疾病进展、19例（33.93%）死亡。结论 斜坡恶性肿瘤发生率较高，可根据适应证选择组织活检术或根治性肿瘤切除术。术前多学科综合评估是明确手术目的、选择个体化手术入路不可或缺的模式；术后积极开展规范化综合治疗，对降低手术相关并发症、改善预后大有裨益。

关键词: 颅底肿瘤, 颅窝,后, 精准医学, 神经外科手术

Abstract:

Background Deep-seated clival varied malignancies represent a surgical challenge because of complex anatomy and surgical procedures posing great risks to adjacent neurovascular structures. To investigate effects of diagnosis and treatment of clival region lesions, review experience of individualized surgical procedures and comprehensive therapy. Methods and Results A total of 56 patients with clival malignancies from July 2007 to December 2018 were included. The patients mainly presented with headache and cranial nerve paresis. After multidisciplinary consultation, radical resection were performed in 45 cases different kinds of surgical approaches including endonasal (15 cases, 33.33%), intraoral accompanied by intraoral and endonasal combined (7 cases, 15.56%), Derome (8 cases, 17.78%), maxillary swing (9 cases, 20%), and infratemporal (6 cases, 13.33%) approaches were chosen according to clinical and radiographic findings, and biopsies were performed in 11 patients to confirm the pathological diagnosis. The primary pathological finding was chordoma (28 cases, 50%). Total resection was achieved in 21 cases (46.67%), and 17 cases (37.78%) underwent subtotal resection. Besides, partial resection was performed in 7 cases (15.56%). The complications developed in 28 patients, including cranial nerve deficits (13 cases, 23.21%), cerebrospinal fluid leakage (14 cases, 25%), intracranial infection (8 cases, 14.29%), pneumonitis (2 cases, 3.57%), postoperative hematoma (2 cases, 3.57%), and mental disorder (1 cases, 1.79%). Adjuvant chemoradiotherapy was provided by the oncologists depending on the pathological findings. The average follow-up was 59.90 months, but 8 patients (14.29%) were lost. The progression-free survival was achieved in 16 patients (28.57%), whereas the disease progression occurred in 13 patients (23.21%). The mortality was 33.93% (19/56). Conclusions Although malignancies occupy a larger subset of clival tumors, not all are needed to be radically resected. Our preliminary results suggest that multidisciplinary consultation is indispensable to clarify the therapeutic purpose and provide individualized surgical approaches. Postoperative comprehensive therapy may reduce the surgical complications and improve the outcomes of patients.

Key words: Skull base neoplasms, Cranial fossa,posterior, Precision medicine, Neurosurgical procedures