Liver tumors in children

Primary liver tumors account for 1-2% of all malignant neoplasms in childhood.

Liver tumors in children

Malignant	Benign
Hepatoblastoma	Hemangioma
Hepatocellular carcinoma	Hamartoma
Rhabdomyosarcoma	Hemanpuendothelioma
Angiosarcoma	Cysts (simple)
Mesenchymal sarcoma	Adenoma

Among malignant tumors, hepatoblastoma and hepatocellular carcinoma are the most common. Several congenital anomalies are known that increase the risk of liver tumors: hemihypertrophy, congenital agenesis of the kidneys or adrenal glands, Wiedemann-Beckwith syndrome (organomegaly, omphalocele, macroglossia, hemihypertrophy), Meckel's diverticulum. The following diseases also increase the risk of liver tumors.

• Cirrhosis:
  • familial cholestatic cirrhosis of childhood;
  • biliary cirrhosis due to biliary atresia;
  • cirrhosis in giant cell hepatitis.
• Metabolic disorders:
  • hereditary tyrosinemia;
  • Gierke's disease;
  • congenital cystinuria combined with hemihypertrophy;
  • a1-antitrypsin deficiency.
• Effects of drugs:
  • androgens;
  • methotrexate.
• Infectious diseases:
  • chronic viral hepatitis B and C.
• Other diseases and conditions:
  • familial adenomatous polyposis.

All patients undergo liver function assessment (determination of transaminase activity, concentration of cholestasis markers, determination of protein synthesis function indicators, blood coagulation parameters). Blood is tested for markers of viral hepatitis (primarily B and C).

Stages of liver tumors in children

Staging takes into account the residual tumor volume after surgical excision.

• Stage I. Completely removed tumor in the absence of metastases.
• Stage II. Microscopically incompletely removed tumor, no metastases; tumor rupture during surgery.
• Stage III. Macroscopically incompletely removed tumor or involvement of regional lymph nodes; absence of metastases.
• Stage IV. Presence of distant metastases.

Treatment of liver tumors in children

Surgical removal of liver tumor is an integral part of effective treatment. In addition to resection of the tumor itself, surgical excision of single metastatic foci in the lungs and brain is effective.

Preoperative chemotherapy can reduce the tumor size, which facilitates its more complete excision. In addition, chemotherapy reduces the risk of intraoperative complications. Adjuvant chemotherapy for hepatoblastoma is performed after complete excision of the tumor in the amount of four courses using cisplatin, vincristine and doxorubicin. In case of completely removed hepatocellular carcinoma, the recommendations are general - repeated courses of chemotherapy with cisplatin and doxorubicin. There are reports of the successful use of chemotherapy in the presence of metastatic foci in the lungs. Chemotherapy can also be used for palliative purposes. Hepatoblastoma is more sensitive to chemotherapy than hepatocellular carcinoma.

The most commonly used chemotherapy regimens include doxorubicin, cisplatin, vincristine, and fluorouracil. In stages III-IV and in case of incomplete tumor resection, high-dose cisplatin in combination with etoposide may be used. Alternative therapy in these cases is tumor chemoembolization via the hepatic artery or orthotopic liver transplantation.

The role of radiation therapy is limited to cases of incomplete tumor removal. As a rule, the effective radiation dose exceeds the radiation tolerance of liver tissue. Irradiation administered immediately after surgery significantly slows down regenerative processes in the liver.

Liver transplantation is a highly effective method of treating tumors. Currently, the 5-year post-transplant survival rate for hepatoblastoma is over 80%, and for hepatocellular carcinoma - about 65%. Risk factors for relapse in the post-transplant period include tumor size, lymph node involvement, presence of distant metastases, tumor growth into vessels, male gender. In hereditary tyrosinemia and familial cholestatic cirrhosis, liver transplantation should be performed as early as possible before the development of severe organ dysfunction and tumor occurrence.

Treatment of recurrent hepatoblastoma is successful provided that they are radically removed. The question of treatment tactics depends on many factors and is decided individually. In case of recurrent hepatocellular cancer, the prognosis is extremely unfavorable.

Forecast

The prognosis for liver tumors is determined by the radicality of surgical treatment and the histological variant.

The overall 2-year survival rate for stages I-II hepatoblastoma is 90%, for stage III - 60%, for stage IV - 20%. The prognosis for hepatocellular carcinoma at stages III-IV is extremely unfavorable.

Favorable histological variants of liver tumors include hepatoblastoma with a fetal histological structure and fibrolamellar carcinoma; unfavorable variants include hepatoblastoma with an embryonic histological structure and hepatocellular carcinoma.