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小儿肝细胞癌的手术策略评估及长期疗效。

Evaluation of surgical strategies and long-term outcomes in pediatric hepatocellular carcinoma.

机构信息

Department of Pediatric Surgery, Faculty of Medicine, Ege University, Izmir, Turkey.

Department of Pediatric Oncology, Faculty of Medicine, Ege University, Izmir, Turkey.

出版信息

Pediatr Surg Int. 2024 May 31;40(1):144. doi: 10.1007/s00383-024-05721-0.

Abstract

PURPOSE

Hepatocellular carcinoma (HCC), the second most common pediatric malignant liver tumor after hepatoblastoma, represents 1% of all pediatric tumors.

METHODS

A retrospective study was conducted on children with HCC treated at our center from March 2002 to October 2022, excluding those with inadequate follow-up or records. Demographic data, initial complaints, alpha-fetoprotein (AFP) values, underlying disease, size and histopathological features of the masses, chemotherapy, and long-term outcomes were analyzed.

RESULTS

Fifteen patients (8 boys, 7 girls) with a mean age of 11.4 ± 4.1 years (0.8-16.4 years) were analyzed. The majority presented with abdominal pain, with a median AFP of 3.9 ng/mL. Hepatitis B cirrhosis in one patient (6.6%) and metabolic disease (tyrosinemia type 1) in two patients (13.3%) were the underlying diseases. Histopathological diagnoses were fibrolamellar HCC (n:8; 53.3%), HCC (n:6; 40%). Four of the 15 patients underwent liver transplantation, and 9 underwent surgical resection. Due to late diagnosis, two patients were considered inoperable (13.3%). The survival rate for the four patients who underwent liver transplantation was found to be 75%.

CONCLUSION

Surgical treatment of various variants of HCC can be safely performed in experienced centers with a multidisciplinary approach, and outcomes are better than in adults.

摘要

目的

肝细胞癌(HCC)是仅次于肝母细胞瘤的第二大常见儿童肝脏恶性肿瘤,占所有儿童肿瘤的 1%。

方法

对 2002 年 3 月至 2022 年 10 月在我院接受治疗的 HCC 患儿进行回顾性研究,排除随访资料不全或无记录的患儿。分析患儿的人口统计学资料、首发症状、甲胎蛋白(AFP)值、基础疾病、肿瘤大小和组织病理学特征、化疗以及长期预后。

结果

分析了 15 例(8 男,7 女)平均年龄为 11.4±4.1 岁(0.8-16.4 岁)的患儿。大多数患儿以腹痛为首发症状,中位 AFP 值为 3.9ng/mL。1 例(6.6%)患儿存在乙型肝炎肝硬化,2 例(13.3%)存在代谢疾病(酪氨酸血症 1 型)。组织病理学诊断为纤维板层 HCC(n=8;53.3%)和 HCC(n=6;40%)。15 例患儿中,4 例行肝移植,9 例行手术切除。由于诊断较晚,2 例患儿(13.3%)被认为无法手术。行肝移植的 4 例患儿的存活率为 75%。

结论

对于有经验的多学科中心,各种类型 HCC 的手术治疗是安全的,且结局优于成人。

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