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内镜经鼻手术治疗颅底软骨肉瘤的作用:单机构 19 例回顾性分析。

Role of endoscopic transnasal surgery for skull base chondrosarcoma: a retrospective analysis of 19 cases at a single institution.

机构信息

Departments of1Neurosurgery and.

2Otorhinolaryngology, University of Tokyo Hospital, Tokyo, Japan.

出版信息

J Neurosurg. 2018 May;128(5):1438-1447. doi: 10.3171/2017.1.JNS162000. Epub 2017 Jul 7.

Abstract

OBJECTIVE Skull base chondrosarcoma is one of the most intractable tumors because of its aggressive biological behavior and involvement of the internal carotid artery and cranial nerves (CNs). One of the most accepted treatment strategies for skull base chondrosarcoma has been surgical removal of the tumor in conjunction with proactive extensive radiation therapy (RT) to the original tumor bed. However, the optimal strategy has not been determined. The goal of this study was to evaluate the early results of endoscopic transnasal surgery (ETS). METHODS The authors retrospectively analyzed 19 consecutive patients who underwent ETS at their institution since 2010. Adjuvant stereotactic radiosurgery (SRS) was performed only for the small residual tumors that were not resected to avoid critical neurological complications. Histological confirmation and evaluation of the MIB-1 index was performed in all cases. The Kaplan-Meier method was used to determine the actuarial rate of tumor-free survival. RESULTS The median tumor volume and maximal diameter were 14.5 cm (range 1.4-88.4 cm) and 3.8 cm (range 1.5-6.7 cm), respectively. Nine patients (47%) had intradural extension of the tumor. Gross-total resection was achieved in 15 (78.9%) of the 19 patients, without any disabling complications. In 4 patients, the surgery resulted in subtotal (n = 2, 11%) or partial (n = 2, 11%) resection because the tumors involved critical structures, including the basilar artery or the lower CNs. These 4 patients were additionally treated with SRS. The median follow-up duration was 47, 28, and 27 months after the diagnosis, ETS, and SRS, respectively. In 1 patient with an anterior skull base chondrosarcoma, the tumor relapsed in the optic canal 1 year later and was treated with a second ETS. Favorable tumor control was achieved in all other patients. The actuarial tumor control rate was 93% at 5 years. At the final follow-up, all patients were alive and able to perform independent activities of daily living without continuous neurological sequelae. CONCLUSIONS These preliminary results suggest that ETS can achieve sufficient radical tumor removal, resulting in comparative resection rates with fewer neurological complications to those in previous reports. Although the follow-up periods of these cases were relatively short, elective SRS to the small tumor remnant may be rational, achieving successful tumor control in some cases, instead of using proactive extensive RT. Thus, the addition of RT should be discussed with each patient, after due consideration of histological grading and biological behavior. To determine the efficacy of this strategy, a larger case series with a longer follow-up period is essential. However, this strategy may be able to establish evidence in the management of skull base chondrosarcoma, providing less-invasive and effective options as an initial step of treatment.

摘要

目的

颅底软骨肉瘤因其侵袭性的生物学行为以及对颈内动脉和颅神经(CNs)的累及而成为最棘手的肿瘤之一。颅底软骨肉瘤最被接受的治疗策略之一是手术切除肿瘤,并结合对原肿瘤床的积极广泛放疗(RT)。然而,最佳策略尚未确定。本研究的目的是评估内镜经鼻手术(ETS)的早期结果。

方法

作者回顾性分析了 2010 年以来在其机构接受 ETS 的 19 例连续患者。对于未能切除的小残留肿瘤,仅行立体定向放射外科(SRS)辅助治疗,以避免严重的神经并发症。所有病例均行组织学证实和 MIB-1 指数评估。采用 Kaplan-Meier 法计算无瘤生存的累积率。

结果

肿瘤体积中位数和最大直径分别为 14.5cm(范围 1.4-88.4cm)和 3.8cm(范围 1.5-6.7cm)。9 例(47%)肿瘤有硬脑膜内延伸。19 例患者中,15 例(78.9%)实现了大体全切除,无任何致残性并发症。由于肿瘤累及包括基底动脉或较低的 CNs 在内的关键结构,4 例(21%)患者的手术导致次全(2 例,11%)或部分(2 例,11%)切除。这 4 例患者另外接受了 SRS 治疗。诊断、ETS 和 SRS 后的中位随访时间分别为 47、28 和 27 个月。1 例前颅底软骨肉瘤患者 1 年后肿瘤在视神经管复发,行第二次 ETS 治疗。所有其他患者均获得了良好的肿瘤控制。5 年时肿瘤控制的累积率为 93%。在最后一次随访时,所有患者均存活,能够独立进行日常生活活动,无持续的神经后遗症。

结论

这些初步结果表明,ETS 可以实现足够的根治性肿瘤切除,与既往报道相比,具有相似的切除率,且神经并发症较少。尽管这些病例的随访时间相对较短,但对小肿瘤残余物选择性行 SRS 可能是合理的,可以在某些病例中成功控制肿瘤,而无需采用积极广泛的 RT。因此,应在考虑组织学分级和生物学行为后,与每位患者讨论 RT 的附加问题。为了确定该策略的疗效,需要进行具有更长随访时间的更大病例系列研究。然而,该策略可能能够为颅底软骨肉瘤的治疗提供依据,作为初始治疗步骤提供侵袭性较小且有效的选择。

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