芬兰儿童肝脏恶性肿瘤手术治疗的发病率及长期预后

Incidence and long-term outcomes of surgically treated childhood hepatic malignancies in Finland.

作者信息

Tiusanen Toivo, Hukkinen Maria, Leskinen Outi, Soini Tea, Kanerva Jukka A, Jahnukainen Timo, Mäkisalo Heikki, Heikinheimo Markku, Pakarinen Mikko P

机构信息

Pediatric Liver and Gut Research Group, Children's Hospital, Helsinki University Hospital, University of Helsinki, Helsinki, Finland.

Pediatric Research Center, Children's Hospital, Helsinki University Hospital, University of Helsinki, Helsinki, Finland.

出版信息

Acta Paediatr. 2020 Feb;109(2):404-414. doi: 10.1111/apa.14952. Epub 2019 Aug 25.

DOI:10.1111/apa.14952

PMID:31350767

Abstract

AIM

To analyse incidence, treatment and outcomes of paediatric liver malignancies in Finland during 1987-2017.

METHODS

Medical records and national cancer registry data of 47 children with liver malignancies were reviewed. Survival was calculated with the Kaplan-Meier method.

RESULTS

During follow-up, liver malignancy incidence remained stable at 1.1:10 . Altogether, 42 patients with hepatoblastoma (n = 24), hepatocellular carcinoma (n = 11) and undifferentiated embryonal sarcoma (n = 7) underwent surgery at median age 4.6 (interquartile range, 2.0-9.6) years and were followed up for 13 (7.0-19) years. Cumulative 5-year survival was 86% for hepatoblastoma, 41% for hepatocellular carcinoma and 67% for undifferentiated embryonal sarcoma. Five-year survival was decreased among hepatoblastoma patients aged ≥ 2.4 years (73% versus 100%, P = .040), with PRETreatment EXTent of disease IV (PRETEXT, 60% vs 100%, P = .004), and with recurrent disease (67% vs 88%, P = .029). Recurrent/residual disease associated with decreased 5-year survival in hepatocellular carcinoma (0% vs 83%, P = .028). Survival was similar among 19 transplanted and 23 resected patients. In total, 14 deaths occurred either for the underlying malignancy (n = 8), adverse effects of chemotherapy (n = 5) or unrelated reasons (n = 1).

CONCLUSION

Outcomes for PRETEXT I-III hepatoblastoma and un-metastasized hepatocellular carcinoma were encouraging. Adverse effects of chemotherapy significantly contributed to mortality.

摘要

目的

分析1987年至2017年芬兰儿童肝脏恶性肿瘤的发病率、治疗方法及预后情况。

方法

回顾了47例肝脏恶性肿瘤患儿的病历和国家癌症登记数据。采用Kaplan-Meier方法计算生存率。

结果

在随访期间，肝脏恶性肿瘤的发病率保持稳定，为1.1:10。共有42例肝母细胞瘤（n = 24）、肝细胞癌（n = 11）和未分化胚胎性肉瘤（n = 7）患者接受了手术，中位年龄为4.6岁（四分位间距，2.0 - 9.6岁），并随访了13年（7.0 - 19年）。肝母细胞瘤的5年累积生存率为86%，肝细胞癌为41%，未分化胚胎性肉瘤为67%。年龄≥2.4岁的肝母细胞瘤患者5年生存率降低（73%对100%，P = .040），疾病分期为IV期（PRETEXT，60%对100%，P = .004），以及复发疾病（67%对88%，P = .029）。复发/残留疾病与肝细胞癌5年生存率降低相关（0%对83%，P = .028）。19例移植患者和23例切除患者的生存率相似。共有14例死亡，原因包括潜在恶性肿瘤（n = 8）、化疗不良反应（n = 5）或无关原因（n = 1）。