Department of Neurological Surgery, Asan Medical Center, University of Ulsan College of Medicine, 88 Olympic-ro 43-gil, Songpa-gu, Seoul, 05505, Korea.
J Neurooncol. 2019 Aug;144(1):147-154. doi: 10.1007/s11060-019-03213-z. Epub 2019 Jun 14.
The growth rate and natural history of untreated hemangioblastomas remain unclear. This study investigated the natural history of untreated intracranial hemangioblastomas and predictors of tumor growth using volumetric assessment.
This study retrospectively enrolled 31 patients with untreated hemangioblastomas between 2004 and 2017 who were followed up for at least 12 months. The 31 patients had a total of 52 hemangioblastomas.
The 31 patients included 11 (35.5%) men and 20 (64.5%) women, of mean age 42.5 years. Seventeen (54.8%) patients were genetically diagnosed with Von Hippel-Lindau (VHL) disease. Of the 52 lesions, 33 (63.5%) grew during the follow-up period, whereas 19 (36.5%) remained stable. Overall mean actual growth rate (AGR) was 1.94 cm/year, 2.38 cm/year in the VHL and 1.79 cm/year in the non-VHL group (p = 0.31). Overall mean relative growth rate (RGR) was 21%/year, 26%/year in the VHL and 19%/year in the non-VHL group. Time to 50% treatment probability was 34 months. The 1, 3, 5, and 7-year treatment probabilities were 11.5%, 50.1%, 52.7%, and 73%, respectively. The presence of only symptomatic lesions was significantly predictive of the growth of intracranial hemangioblastoma (odds ratio: 5.0, p = 0.02).
The overall growth rate of intracranial hemangioblastoma was faster than that of other benign intracranial tumors, with symptomatic lesions being the only meaningful predictor of tumor growth. Because of their rapid growth rate and high probability of treatment, a wait and scan management strategy should be carefully applied to intracranial hemangioblastomas.
未经治疗的血管母细胞瘤的生长速度和自然史尚不清楚。本研究通过体积评估，调查了未经治疗的颅内血管母细胞瘤的自然史和肿瘤生长的预测因素。
本研究回顾性纳入了 2004 年至 2017 年间的 31 例未经治疗的血管母细胞瘤患者，这些患者的随访时间至少为 12 个月。这 31 例患者共有 52 个血管母细胞瘤。
这 31 例患者包括 11 例（35.5%）男性和 20 例（64.5%）女性，平均年龄为 42.5 岁。17 例（54.8%）患者经基因诊断为 Von Hippel-Lindau（VHL）病。在 52 个病变中，33 个（63.5%）在随访期间生长，而 19 个（36.5%）保持稳定。总体实际生长率（AGR）为 1.94cm/年，VHL 组为 2.38cm/年，非 VHL 组为 1.79cm/年（p=0.31）。总体平均相对生长率（RGR）为 21%/年，VHL 组为 26%/年，非 VHL 组为 19%/年。达到 50%治疗概率的时间为 34 个月。1、3、5 和 7 年的治疗概率分别为 11.5%、50.1%、52.7%和 73%。仅有症状性病变的存在与颅内血管母细胞瘤的生长显著相关（优势比：5.0，p=0.02）。
颅内血管母细胞瘤的总体生长速度快于其他良性颅内肿瘤，仅有症状性病变是肿瘤生长的唯一有意义的预测因素。由于其生长速度快且治疗概率高，应仔细应用等待和扫描的管理策略来处理颅内血管母细胞瘤。
