Surgery of focal liver lesions in children: conserving surgery or transplantation?

Hepatic tumors account for 1–4% of the solid tumors in children.

Aim. Results of surgery treatment assessment for pediatric liver tumors.

Material and methods. From April 2008 to December 2016, 97 pediatric patients (2 months – 17 years old) (median – 23.7; 25th and 75th quartiles – 13–80 months) with a variety of focal liver lesions underwent surgery at Petrovsky National Research Center of Surgery. Malignant tumors (n=74; 76.3%) were presented by hepatoblastoma (n=65; 67%), hepatocellular carcinoma (n=3; 3%), undifferentiated (embryonal) sarcoma (n=2; 2%), malignant rhabdoid tumor (n=2; 2%) and rhabdomyosarcoma (n=2; 2%). Benign lesions or neoplasms (n=23; 23.7%) were presented by focal nodular hyperplasia (n=7; 8%), mesenhymal hamartoma (n=5; 5%), hepatocellular adenoma (n=3; 3%), infantile hemangioendothelioma (n=2; 2%), benign teratoma (n=2; 2%), cavernous hemangioma (n=1; 1%), inflammatory pseudotumor (n=1; 1%). Two patients (2%) had a parasitic liver disease: cystic and alveolar echinococcosis. Among the revealed tumors predominated hepatoblastoma. 

Results. Patients with hepatoblastoma were classified according to PRETEXT system: PRETEXT I – 5% (n=3), PRETEXT II – 40% (n=26), PRETEXT III – 29% (n=19), PRETEXT IV – 26% (n=17). In 15 children (23.1%) detected lung metastases. Neoadjuvant chemotherapy was performed in 62 cases (95.4%). 8 patients (12.9%) had tumor regression with reduction of the tumor stage, and 6 (9.7%) had increase in tumor progression. Due to the remote metastases in 7 cases lung surgery was performed. Surgical treatment of hepatoblastoma included anatomical resection (lobe- and extended lobectomies – 81.5% (n=53), bisegmentectomies – 6.2% (n=4), and living-donor liver transplantations – 12.3% (n=8). All resections were performed without clamping hepatoduodenal ligament. The volume of blood loss in patients with resection was 15.56±14.00 ml/kg. Tumor free resection edge (R0 resection) was microscopically confirmed in all cases. In the group of malignant tumors hospital mortality was 1% (n=1). The actuarial survival rate (total/recurrence-free) of all patients with hepatoblastoma was, respectively: 1 year 0.95±0.03/0.83±0.05; 3 years 0.88±0.05/0.81±0.05; 5 years 0.88±0.04/0.81±0.05; 7 years 0.88±0.05/0.81±0.05. In patients after resection corresponding figures were higher: 1 year 0.96±0.02/0.88±0.04; 3 years 0.89±0.05/0.86±0.05; 5 years 0.89±0.05/0.86±0.05; 7 years 0.89±0.05/0.86±0.05. In the group with other forms of liver malignancies 2 of 9 patients died in the period from 2 to 30 months due to the initial disease relapse. In groups of benign and parasitic diseases there were no deaths. Conclusion. Surgical methods occupy a leading position in the radical treatment of both benign and malignant focal lesions of liver in children. Long-term results with high survival rates for combined treatment of hepatoblastoma demonstrate broad opportunities for both resection and liver transplantation under condition if optimal chemotherapy protocols are being selected. 

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