Douglas Cohen Department of Paediatric Surgery, The Children's Hospital at Westmead, NSW 2145, Australia.
J Pediatr Surg. 2013 Dec;48(12):2474-8. doi: 10.1016/j.jpedsurg.2013.08.023.
Hepatoblastoma is a rare tumour accounting for approximately 1% of all paediatric malignancies. Hepatoblastoma complicated by pulmonary metastatic disease continues to cause management difficulties due to a lack of robust evidence and treatment guidelines.
This series is the experience of a tertiary paediatric referral centre. Patients were prospectively enlisted, and their charts were retrospectively reviewed.
Thirty-seven patients were treated for hepatoblastoma from 1995 to 2012 inclusive. The overall survival was 34/37(91.9%). Eight patients had lung metastases at diagnosis (LMD) and twenty-nine did not (NLMD). Two-year EFS was 62.5% in the LMD group and 89.3% in the NLMD group (p=0.078). Overall survival in the LMD and NLMD groups was 100% and 89.7%, respectively (p=0.389). Two patients in the LMD group required multiple thoracic resections to achieve cure. Within the NLMD group, two patients developed lung metastases whilst on treatment, and both of these patients died.
In this series, children presenting with lung metastases had a higher risk of relapse but excellent overall survival. However, children who developed pulmonary disease during treatment had a poor prognosis. We advocate aggressive surgical treatment of pulmonary hepatoblastoma to achieve cure.
肝母细胞瘤是一种罕见的肿瘤，约占所有儿科恶性肿瘤的 1%。由于缺乏强有力的证据和治疗指南，肝母细胞瘤合并肺转移疾病仍然导致治疗困难。
本系列是一家三级儿科转诊中心的经验。前瞻性招募患者，并回顾性分析其图表。
1995 年至 2012 年共治疗 37 例肝母细胞瘤患者。总生存率为 34/37（91.9%）。8 例患者在诊断时即有肺转移（LMD），29 例患者无肺转移（NLMD）。LMD 组和 NLMD 组的 2 年 EFS 分别为 62.5%和 89.3%（p=0.078）。LMD 组和 NLMD 组的总生存率分别为 100%和 89.7%（p=0.389）。LMD 组中有 2 例患者需要多次开胸手术才能治愈。NLMD 组中有 2 例患者在治疗过程中发生肺部转移，这 2 例患者均死亡。
在本系列中，出现肺转移的儿童复发风险较高，但总体生存率良好。然而，在治疗过程中发生肺部疾病的儿童预后较差。我们主张积极手术治疗肺部肝母细胞瘤以达到治愈。
