Radiosurgery for symptomatic cavernous malformations in the brainstem

Y. Kida, T. Hasegawa
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Abstract

Purpose: Cavernous malformations are believed to be clinically silent and some are found just incidentally. However, the lesions once become symptomatic with hemorrhage or epilepsy, they are very troublesome because the repeated episodes may happen. Since the majority of symptomatic incidents of the brainstem lesions are hemorrhage, the prompt treatments are required. Microsurgery has a limited indication such as the lesions easy to access and located just beneath the brainstem surface. It is often difficult and risky to undergo surgery, which requires the skills of the microsurgeon to complete these tasks. Methods and cases: Radiosurgery is an alternative to microsurgery, which has been chosen for treating such symptomatic lesions. Because radiosurgery required no special techniques and can be performed by standard radio surgeons with sufficient knowledge. After the brainstem hemorrhage once or twice, radiosurgery was performed with a mean marginal dose of 12.8 Gy. After the radiosurgery, the follow-up studies were performed at the intervals of every 3 to 6 months. Results: Radiological studies with MRI demonstrated a lesion shrinkage approximately in half of the lesions, and the others showed no obvious change. A few lesions caused hemorrhage showing enlargement of the lesion in the association of clinical signs, even after the treatment. Hemorrhage rate before and after the treatment was considerably decreased from 30 %/year/case to 5 %/year/case after the treatment. Conclusion: Radiosurgery with gamma knife reduced the hemorrhage rate significantly and the lesions were smaller in half and the other half showed no remarkable changes. Since the indication for microsurgery is restricted, radiosurgery is the better treatment option for symptomatic CMs in the brainstem. Radiosurgery might be able to change the natural course of this peculiar disease. Abbreviation: CM: Cavernous malformation; CNS: Central nervous system; HR: Hemorrhage rate; PFS: Progression-free-survival. Introduction Cavernous malformations (CM) are one of the vascular anomalies similar to arteriovenous malformation, venous anomaly and capillary telangiectasia in the central nervous system (CNS). Different from the other three, CMs are a very peculiar disease. The majority of them are usually very silent, however, they become symptomatic all of the sudden in association with repeated hemorrhages and frequent epilepsy attacks. Once becoming symptomatic, they are so troublesome and may develop frequent episodes and neurological deterioration. This is true especially when the brainstem lesions become symptomatic, exclusively with hemorrhage. Motor dysfunction, ataxia, or disturbed ocular movement are the main and popular symptoms of brainstem hemorrhages. Since they may often appear repeatedly, the patients require prompt treatment procedures to stop the bleeding. A waitand-see strategy may be taken into account when the microsurgery is difficult because of the location and the patient’s condition. We, Gamma Knife Teams in Japan, have currently treated these symptomatic CMs in the brainstem with gamma knife radiosurgery in the past 30 years. Here in this paper, our treatment results are summarized and consider the strategy of this serious disease. Cases and methods For this retrospective study, cases were collected from nation-wide Gamma Knife Institutes in Japan. There were 139 cases, 85 males and *Correspondence to: Yoshihisa Kida, Scientific Committee of Japanese Gamma Knife Society, Gamma Knife Center in Okuma Hospital, Nagoya, Japan, E-mail: yoshihisa_kida@mac.com
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脑干海绵状血管瘤的放射外科治疗
目的:海绵体畸形被认为是临床无症状的,有些只是偶然发现的。然而,病变一旦出现出血或癫痫的症状,就会因为反复发作而非常麻烦。由于大多数脑干病变的症状是出血,因此需要及时治疗。显微外科手术的适应症有限,如病灶容易接近且位于脑干表面以下。接受手术通常是困难和危险的,这需要显微外科医生的技能来完成这些任务。方法和病例:放射外科是显微手术的替代方法,已被选择用于治疗此类症状性病变。因为放射外科手术不需要特殊的技术,可以由具有足够知识的标准放射外科医生进行。脑干出血1 ~ 2次后行平均边缘剂量12.8 Gy的放射治疗。放疗后每隔3 ~ 6个月进行随访研究。结果:MRI影像学检查显示约一半病变缩小,其余病变无明显变化。少数病变引起出血,表现出病变扩大的临床体征,甚至在治疗后。治疗前后出血率由治疗前的30% /年/例显著下降到治疗后的5% /年/例。结论:伽玛刀放射治疗可明显降低出血率,一半病变缩小,另一半无明显变化。由于显微手术的适应症受到限制,放射手术是脑干症状性CMs的更好治疗选择。放射外科也许能改变这种特殊疾病的自然病程。缩写:CM:海绵状畸形;CNS:中枢神经系统;HR:出血率;PFS: Progression-free-survival。海绵状畸形()是一种类似于中枢神经系统动静脉畸形、静脉畸形和毛细血管扩张的血管异常。与其他三种疾病不同,CMs是一种非常特殊的疾病。他们中的大多数通常很沉默,然而,他们突然出现症状,伴有反复出血和频繁的癫痫发作。一旦出现症状,它们就很麻烦,可能会频繁发作和神经退化。这是真的,特别是当脑干病变出现症状时,只有出血。运动功能障碍、共济失调或眼球运动紊乱是脑干出血的主要和常见症状。由于他们可能经常反复出现,病人需要及时的治疗程序来止血。当显微手术由于位置和患者的状况而难以进行时,可以考虑采取观望策略。我们,日本的伽玛刀小组,在过去的30年里用伽玛刀放射手术治疗脑干的症状性CMs。在此,本文总结了我们的治疗结果,并对这一严重疾病的策略进行了思考。病例与方法本回顾性研究收集了日本全国性伽玛刀研究所的病例。139例,男85例。通讯:日本名古屋大隈医院伽玛刀中心日本伽玛刀学会科学委员会木田义久,E-mail: yoshihisa_kida@mac.com
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