Schnater J M, Kuijper C F, Zsiros J, Heij H A, Aronson D C
Pediatric Surgical Centre, Emma Children's Hospital AMC, Meibergdreef 9, 1105 AZ Amsterdam, The Netherlands.
Eur J Surg Oncol. 2005 Dec;31(10):1160-5. doi: 10.1016/j.ejso.2005.07.012. Epub 2005 Sep 12.
To report 24 years of pre-treatment biopsy and surgical experience in primary liver tumours in children.
Between 1979 and 2003, 53 children presented with a primary liver tumour of whom 48 who underwent surgical resection were evaluated (two died, two were unresectable, and one was transplanted). Biopsy data, per- and post-operative complications, mortality, and survival were retrospectively reviewed.
Benign tumours were diagnosed in eight patients. Surgical resection for a malignant tumour was performed in 40 patients (26 hepatoblastomas (HB), eight hepatocellular carcinomas (HCC) (four had fibrolamellar HCC), three rhabdomyosarcomas (RMS), one neuroblastoma, one non-hodgkin lymphoma (NHL), and one teratoma). Primary resection was performed in one HB, and four HCCs. The cumulative survival without evidence of disease was 73% for HB (median 7 years) and 88% for HCC (median 3.5 years).
The treatment results are comparable with those of larger international series except for HCC. The existing diagnostic pitfalls in differentiating between the various liver malignancies justify the use of a diagnostic biopsy.
报告24年儿童原发性肝肿瘤的治疗前活检及手术经验。
1979年至2003年间,53例儿童患有原发性肝肿瘤,其中48例接受了手术切除(2例死亡,2例无法切除,1例接受移植)。对活检数据、围手术期及术后并发症、死亡率和生存率进行回顾性分析。
8例患者诊断为良性肿瘤。40例患者接受了恶性肿瘤的手术切除(26例肝母细胞瘤(HB),8例肝细胞癌(HCC)(4例为纤维板层型HCC),3例横纹肌肉瘤(RMS),1例神经母细胞瘤,1例非霍奇金淋巴瘤(NHL),1例畸胎瘤)。1例HB和4例HCC进行了一期切除。无疾病证据的累积生存率,HB为73%(中位生存期7年),HCC为88%(中位生存期3.5年)。
除HCC外,治疗结果与国际上更大规模系列研究的结果相当。鉴别各种肝脏恶性肿瘤时现有的诊断缺陷证明了诊断性活检的应用价值。