Lyu Fang, Gan Jinlu, Wang Haijun, Zhao Hongyang, Wang Lei, Zhang Fangcheng
Department of Neurosurgery, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China.
Front Surg. 2022 May 25;9:759163. doi: 10.3389/fsurg.2022.759163. eCollection 2022.
Rare giant vestibular schwannomas (GVSs) invade the temporal bone extensively, which carries unique risks for surgery owing to their complicated relationship with adjacent structures, difficult dissection of the temporal bone, and high risk of complications. The underlying mechanism of this invasive behavior remains unknown.
We report on a 28-year-old woman who presented with typical hearing loss and facial paralysis (House-Brackmann II). Magnetic resonance imaging exhibited a giant mass (∼5.0 cm) in the right cerebellopontine angle (CPA), petrous apex, and middle cranial fossa. Her primary diagnosis was GVS with petrous apex invasion. With the aid of presurgical imaging reconstruction and intraoperative facial nerve monitoring, we adopted a sequential therapeutic strategy, which included microsurgery for the CPA lesion followed by gamma knife radiosurgery (GKRS) for the petrous mass. During follow-up, stable tumor control was achieved with functional preservation of the facial nerve and no other complications. The postoperative immunohistochemical examination demonstrated dramatic intratumoral inflammation, which suggested its potential role in bony erosion. We reviewed the literature of large vestibular schwannoma with a petrous invasion and further discussed its treatment.
Microsurgery remains the top therapeutic strategy for GVS. However, gross total resection with functional preservation of cranial nerves is challenging to achieve once the temporal bone is involved. In this case, we applied a planned and sequential approach of microsurgery and GKRS with a promising outcome, which highlighted this combinational strategy in this rare situation. In addition, pathological examination suggested that intratumoral inflammation might play a role in the bony erosion of GVS. Longer observation and more cases are needed to further investigate its molecular mechanism and treatment plan.
罕见的巨大前庭神经鞘瘤（GVS）广泛侵犯颞骨，由于其与相邻结构的复杂关系、颞骨解剖困难以及并发症风险高，给手术带来了独特的风险。这种侵袭行为的潜在机制尚不清楚。
我们报告了一名28岁女性，她表现出典型的听力损失和面瘫（House-Brackmann II级）。磁共振成像显示右侧小脑脑桥角（CPA）、岩尖和中颅窝有一个巨大肿块（约5.0厘米）。她的初步诊断是伴有岩尖侵犯的GVS。借助术前影像重建和术中面神经监测，我们采用了序贯治疗策略，包括对CPA病变进行显微手术，然后对岩部肿块进行伽玛刀放射外科治疗（GKRS）。在随访期间，实现了稳定的肿瘤控制，面神经功能得以保留，且无其他并发症。术后免疫组化检查显示肿瘤内有显著炎症，提示其在骨质侵蚀中可能发挥的作用。我们回顾了伴有岩部侵犯的大型前庭神经鞘瘤的文献，并进一步讨论了其治疗方法。
显微手术仍然是GVS的首要治疗策略。然而，一旦颞骨受累，要在保留颅神经功能的情况下实现全切具有挑战性。在本病例中，我们应用了显微手术和GKRS的计划性序贯方法，取得了良好的效果，突出了这种联合策略在这种罕见情况下的应用。此外，病理检查表明肿瘤内炎症可能在GVS的骨质侵蚀中起作用。需要更长时间的观察和更多病例来进一步研究其分子机制和治疗方案。
