Moscovici Samuel, Candanedo Carlos, Spektor Sergey, Cohen José E, Kaye Andrew H
Department of Neurosurgery, Hadassah-Hebrew University Medical Center, P.O. Box 12000, 91120, Jerusalem, Israel.
Acta Neurochir (Wien). 2022 May;164(5):1357-1364. doi: 10.1007/s00701-021-04828-w. Epub 2021 Apr 3.
Hemangioblastomas (HGBs) are highly vascular benign tumors, commonly located in the posterior fossa, and 80% of them are sporadic. Patients usually present with features of raised intracranial pressure and cerebellar symptoms. HGB can be classified as either mostly cystic or solids. Although the solid component is highly vascularized, aneurysm or hemorrhagic presentation is rarely described, having catastrophic results.
We identified 32 consecutive patients with posterior fossa HBG who underwent surgery from 2008 through 2020 at our medical center. Tumors were classified as predominantly cystic or solid according to radiological features. Resection was defined as gross total (GTR) or subtotal (STR).
During the study period, 32 posterior fossa HGBs were resected. There were 26 cerebellar lesions and 4 medullar lesions, and in 2 patients, both structures were affected. Predominant cystic tumors were seen in 15 patients and solids in 17. Preoperative digital subtraction angiography (DSA) was performed in 8 patients with solid tumors, and 4 showed tumor-related aneurysms. Embolization of the tumors was performed in 6 patients, including the four tumor-related aneurysms. GTR was achieved in 29 tumors (91%), and subtotal resection in 3 (9%). Three patients had postoperative lower cranial nerve palsy. Functional status was stable in 5 patients (16%), improved in 24 (75%), and 3 patients (9%) deteriorated. One patient died 2 months after the surgery. Two tumors recurred and underwent a second surgery achieving GTR. The mean follow-up was 42.7 months (SD ± 51.0 months).
Predominant cystic HGB is usually easily treated as the surgery is straightforward. Those with a solid predominance present a more complex challenge sharing features similar to arteriovenous malformations. Given the important vascular association of solid predominance HGB with these added risk factors, the preoperative assessment should include DSA, as in arteriovenous malformations, and endovascular intervention should be considered before surgery.
血管母细胞瘤(HGBs)是高度血管化的良性肿瘤,通常位于后颅窝,其中80%为散发性。患者通常表现为颅内压升高和小脑症状。HGB可分为主要为囊性或实性。虽然实性成分血管高度丰富,但动脉瘤或出血表现很少被描述,却会产生灾难性后果。
我们确定了2008年至2020年在我们医疗中心接受手术的32例连续的后颅窝HBG患者。根据放射学特征将肿瘤分为主要为囊性或实性。切除定义为全切除(GTR)或次全切除(STR)。
在研究期间,32例后颅窝HGBs被切除。有26例小脑病变和4例延髓病变,2例患者两个结构均受累。15例患者为主要囊性肿瘤,17例为实性肿瘤。8例实性肿瘤患者术前行数字减影血管造影(DSA),4例显示肿瘤相关动脉瘤。6例患者进行了肿瘤栓塞,包括4例肿瘤相关动脉瘤。29例肿瘤(91%)实现了GTR,3例(9%)次全切除。3例患者术后出现下颅神经麻痹。5例患者(16%)功能状态稳定,24例(75%)改善,3例患者(9%)恶化。1例患者术后2个月死亡。2例肿瘤复发并接受了第二次手术,实现了GTR。平均随访42.7个月(标准差±51.0个月)。
主要为囊性的HGB通常易于治疗,因为手术简单直接。以实性为主的HGB带来更复杂的挑战,具有与动静脉畸形相似的特征。鉴于以实性为主的HGB与这些额外风险因素的重要血管关联,术前评估应包括DSA,如同动静脉畸形一样,并且在手术前应考虑血管内介入。