Bründl Elisabeth, Schödel Petra, Ullrich Odo-Winfried, Brawanski Alexander, Schebesch Karl-Michael
Department of Neurosurgery, University Medical Center Regensburg, Germany.
Surg Neurol Int. 2014 Sep 22;5:138. doi: 10.4103/2152-7806.141469. eCollection 2014.
Hemangioblastomas (HBLs) are benign neoplasms that contribute to 1-2.5% of intracranial tumors and 7-12% of posterior fossa lesions in adult patients. HBLs either evolve hereditarily in association with von Hippel-Lindau disease (vHL) or, more prevalently, as solitary sporadic tumors. Only few authors have reported on the clinical presentation and the neurological outcome of HBL.
We retrospectively analyzed the clinical, radiological, surgical, and histopathologic records of 24 consecutive patients (11 men, 13 women; mean age 51.3 years) with HBL of the posterior cranial fossa, who had been treated at our center between 2001 and 2012. We reviewed the current literature, and discussed our findings in the context of previous publications on HBL. The study protocol was approved by the local ethics committee (14-101-0070).
Mean time to diagnosis was 14 weeks. The extent of resection (EOR) was total in 20 and near total in 4 patients. Four patients required revision within 24 h because of relevant postoperative bleeding. One patient died within 14 days. One patient required permanent shunting. At discharge, 75% of patients [n = 18, modified Rankin scale (mRS) 0-1] showed no or at least resolved symptoms. Mean follow-up was 21 months. Two recurrences were detected during follow-up.
In comparison to other benign entities of the posterior fossa, time to diagnosis was significantly shorter for HBL. This finding indicates the rather aggressive biological behavior of these excessively vascularized tumors. In our series, however, the rate of complete resection was high, and morbidity and mortality rates were within the reported range.
成血管细胞瘤（HBLs）是良性肿瘤，占成年患者颅内肿瘤的1 - 2.5%，后颅窝病变的7 - 12%。HBLs要么与冯·希佩尔-林道病（vHL）相关联而遗传发生，要么更常见地作为孤立性散发性肿瘤出现。仅有少数作者报道过HBL的临床表现和神经学转归。
我们回顾性分析了2001年至2012年期间在我们中心接受治疗的24例（11例男性，13例女性；平均年龄51.3岁）后颅窝HBL患者的临床、放射学、手术和组织病理学记录。我们查阅了当前文献，并结合之前关于HBL的出版物讨论了我们的研究结果。该研究方案已获得当地伦理委员会批准（14 - 101 - 0070）。
平均诊断时间为14周。20例患者实现全切，4例患者近全切。4例患者因术后相关出血在24小时内需要再次手术。1例患者在14天内死亡。1例患者需要永久性分流。出院时，75%的患者（n = 18，改良Rankin量表[mRS] 0 - 1）无或至少症状缓解。平均随访时间为21个月。随访期间发现2例复发。
与后颅窝其他良性病变相比，HBL的诊断时间明显更短。这一发现表明这些血管过度丰富的肿瘤具有相当侵袭性的生物学行为。然而，在我们的系列研究中，全切率较高，发病率和死亡率在报道范围内。
