Wang Qiguang, Zhang Si, Cheng Jian, Liu Wenke, Hui Xuhui
Department of Neurosurgery, West China Hospital of Sichuan University, Chengdu, Sichuan, China.
Department of Neurosurgery, West China Hospital of Sichuan University, Chengdu, Sichuan, China.
World Neurosurg. 2017 Dec;108:143-150. doi: 10.1016/j.wneu.2017.08.155. Epub 2017 Sep 1.
Hemangioblastomas with enhanced cyst walls represent a rare radiologic presentation of hemangioblastomas with poor understandings. We aimed to summarize the clinical and radiologic features, important differential diagnosis, surgical strategy, and clinical outcome of this rare entity.
From June 2008 to March 2017, 12 patients with cystic hemangioblastomas presenting with enhanced wall thickness on MRI were treated in our department. The clinical presentations, radiologic investigations, surgical treatment, neurologic outcome, and recurrence rate were evaluated. Important preoperative differential diagnosis and surgical strategy of this entity were discussed.
Twelve patients with cystic hemangioblastomas presenting with an enhanced cyst wall on magnetic resonance imaging were analyzed retrospectively. There were 5 male and 7 female subjects, with a mean age of 41.4 years (range, 13-78 years) and an average duration of symptoms before diagnosis of 2.23 months (range, 0.5-8.0 months). Radiologically, enhancement of both tumoral nodule and cyst were observed in 8 patients, while pure ring-enhanced cyst without typical tumoral nodule was found in 4 patients. Histopathologic examination confirmed the diagnosis of hemangioblastomas, and the enhanced cyst wall and mural nodule shared the same histopathologic pattern. Postoperative complications occurred in only 1 patient with postoperative cerebellar hemorrhage. During follow-up, 8 patients achieved favorable neurologic outcomes (Karnofsky score: 100) without recurrence; however, 4 patients experienced local tumor recurrence after the initial surgery.
Hemangioblastomas with enhanced cyst wall possess distinctive radiologic features, and they are frequently misdiagnosed preoperatively. Favorable tumor control can be achieved only when gross total resection of both the tumor nodule and cyst wall are performed. Close follow-up is necessary because of the high recurrence rate in this subset of hemangioblastomas.
囊肿壁强化的血管母细胞瘤是一种罕见的影像学表现，目前对其了解甚少。我们旨在总结这一罕见实体的临床和影像学特征、重要鉴别诊断、手术策略及临床结局。
2008年6月至2017年3月，我科收治了12例磁共振成像（MRI）显示囊肿壁厚度强化的囊性血管母细胞瘤患者。对其临床表现、影像学检查、手术治疗、神经功能结局及复发率进行评估。讨论了该实体重要的术前鉴别诊断及手术策略。
回顾性分析12例磁共振成像显示囊肿壁强化的囊性血管母细胞瘤患者。其中男性5例，女性7例，平均年龄41.4岁（范围13 - 78岁），诊断前症状平均持续时间2.23个月（范围0.5 - 8.0个月）。影像学上，8例患者肿瘤结节和囊肿均有强化，4例患者为无典型肿瘤结节的单纯环形强化囊肿。组织病理学检查确诊为血管母细胞瘤，强化的囊肿壁和壁结节具有相同的组织病理学模式。术后仅1例患者出现小脑出血并发症。随访期间，8例患者神经功能结局良好（卡诺夫斯基评分：100），无复发；然而，4例患者在初次手术后出现局部肿瘤复发。
囊肿壁强化的血管母细胞瘤具有独特的影像学特征，术前常被误诊。只有当肿瘤结节和囊肿壁均实现肉眼下全切时，才能实现良好的肿瘤控制。由于该亚型血管母细胞瘤复发率高，因此需要密切随访。
