J Neurosurg. 2022 Apr 29;137(6):1733-1741. doi: 10.3171/2022.2.JNS22166. Print 2022 Dec 1.
Giant cerebral cavernous malformations (GCCMs) are rare vascular malformations. Unlike for tumors and aneurysms, there is no clear definition of a "giant" cavernous malformation (CM). As a result of variable definitions, working descriptions and outcome data of patients with GCCM are unclear. A new definition of GCCM related to surgical outcomes is needed.
An institutional database was searched for all patients who underwent resection of CMs > 1 cm in diameter. Patient information, surgical technique, and clinical and radiographic outcomes were assessed. A systematic review was performed to augment an earlier published review.
In the authors' institutional cohort of 183 patients with a large CM, 179 with preoperative and postoperative modified Rankin Scale (mRS) scores were analyzed. A maximum CM diameter of ≥ 3 cm was associated with greater risk of severe postoperative decline (≥ 2-point increase in mRS score). After adjustment for age and deep versus superficial location, size ≥ 3 cm was strongly predictive of severe postoperative decline (OR 4.5, 95% CI 1.2-16.9). A model with CM size and deep versus superficial location was developed to predict severe postoperative decline (area under the receiver operating characteristic curve 0.79). Thirteen more patients with GCCMs have been reported in the literature since the most recent systematic review, including some patients who were treated earlier and not discussed in the previous review.
The authors propose that cerebral CMs with a diameter ≥ 3 cm be defined as GCCMs on the basis of the inflection point for functional and neurological outcomes. This definition is in line with the definitions for other giant lesions. It is less exclusive than earlier definitions but captures the rarity of these lesions (approximately 1% incidence) and variation in outcomes. GCCMs remain operable with potentially favorable outcomes. The term "giant" is not meant to deter or contraindicate surgery.
巨大脑海绵状血管畸形（GCCM）是一种罕见的血管畸形。与肿瘤和动脉瘤不同，对于“巨大”海绵状血管畸形（CM）并没有明确的定义。由于定义不同，GCCM 患者的工作描述和结果数据并不明确。因此，需要一个与手术结果相关的新的 GCCM 定义。
对所有接受直径>1cm 的 CM 切除术的患者进行了机构数据库搜索。评估了患者信息、手术技术以及临床和影像学结果。进行了系统评价，以补充早期发表的综述。
在作者机构的 183 例大型 CM 患者队列中，分析了 179 例具有术前和术后改良 Rankin 量表（mRS）评分的患者。CM 最大直径≥3cm 与术后严重恶化的风险增加相关（mRS 评分增加≥2 分）。在调整年龄和深浅位置后，大小≥3cm 强烈预测术后严重恶化（OR 4.5，95%CI 1.2-16.9）。建立了一个包含 CM 大小和深浅位置的模型来预测术后严重恶化（接受者操作特征曲线下面积 0.79）。自最近一次系统评价以来，文献中又报道了 13 例 GCCM 患者，其中包括一些更早接受治疗且未在之前的综述中讨论的患者。
作者提出，根据功能和神经结局的转折点，将直径≥3cm 的大脑 CM 定义为 GCCM。这个定义与其他巨大病变的定义一致。它没有早期定义那么排他性，但捕捉到了这些病变的罕见性（约 1%的发病率）和结局的变化。GCCM 仍然可以通过手术治疗，并且可能有良好的预后。“巨大”一词并不是为了阻止或禁忌手术。
