Han Bo, Zhang Liang, Jia Wenqing
Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, Beijing, China.
National Cancer Center/National Clinical Research Center for Cancer/Hebei Cancer Hospital, Chinese Academy of Medical Sciences, Langfang, China.
Neurospine. 2023 Mar;20(1):343-352. doi: 10.14245/ns.2244970.485. Epub 2023 Mar 31.
Spinal hemangioblastomas (HBs) are a rare pathology, especially in the pediatric population. The natural history and long-term outcomes of pediatric patients with spinal HBs remain unclear due to their scarcity.
A retrospective review of the clinical data and treatment outcomes of children with spinal HBs in our institution from 2012 to 2021 was conducted.
Thirty-nine pediatric patients were included, with an average age of 15.9 ± 2.9 years (range, 8-18 years), and 51.3% were female. Children were more likely to have von Hippel-Lindau (VHL) disease (p < 0.001), a family history of VHL (p < 0.001), multiple symptoms (p = 0.006), a shorter duration of symptoms (p < 0.001), and a larger lesion size (p = 0.004) and volume (p = 0.008) than their adult counterparts. The VHL-associated group of patients was more likely to present with multiple symptoms (p = 0.026), have a family history of VHL (p < 0.001), have multiple HBs (p < 0.001) and have synchronous intracranial lesions (p < 0.001) than the sporadic group. After surgery, 15 patients (38.5%) showed improved clinical outcomes, 17 patients (43.6%) remained unchanged, 4 patients (10.2%) worsened, and 3 patients (7.7%) died of tumor progression. During follow-up, there was a high rate of recurrence and repeated surgery, especially for children in the VHL-associated group.
Pediatric patients with spinal HBs appear to have a higher relapse risk than their adult counterparts. Therefore, life-long follow-up of these patients is necessary, especially for VHL-associated cases. Surgery can benefit children with HBs and should be considered early to avoid irreversible neurological deterioration.
脊髓血管母细胞瘤（HBs）是一种罕见的病理类型，在儿科人群中尤为少见。由于其病例稀缺，小儿脊髓HBs患者的自然病史和长期预后仍不明确。
对我院2012年至2021年期间小儿脊髓HBs患者的临床资料和治疗结果进行回顾性分析。
共纳入39例儿科患者，平均年龄15.9±2.9岁（范围8 - 18岁），女性占51.3%。与成人患者相比，儿童更易患冯·希佩尔 - 林道（VHL）病（p < 0.001）、有VHL家族史（p < 0.001）、出现多种症状（p = 0.006）、症状持续时间较短（p < 0.001）、病变大小（p = 0.004）和体积（p = 0.008）更大。与散发组相比，VHL相关组患者更易出现多种症状（p = 0.026）、有VHL家族史（p < 0.001）、有多发性HBs（p < 0.001）和同步颅内病变（p < 0.001）。手术后，15例患者（38.5%）临床结果改善，17例患者（43.6%）无变化，4例患者（10.2%）病情恶化，3例患者（7.7%）死于肿瘤进展。随访期间，复发率和再次手术率较高，尤其是VHL相关组的儿童。
小儿脊髓HBs患者的复发风险似乎高于成人患者。因此，对这些患者进行终身随访是必要的，尤其是VHL相关病例。手术对HBs患儿有益，应尽早考虑以避免不可逆的神经功能恶化。
