Tumors of the liver in children

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

Tumors of the liver in children

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

Among malignant tumors, hepatoblastoma and hepatocellular carcinoma are most common. Several congenital anomalies are known in which there is an increased risk of liver tumors: hemihypertrophy, congenital renal agenesis or adrenal gland syndrome, Wiedemann-Bekuit syndrome (organomegaly, omphalocele, macroglossia, hemihypertrophy), mekkel diverticulum. Below are listed the diseases in which the risk of liver tumors is also increased.

• Cirrhosis of the liver:
  • family cholestatic cirrhosis of childhood;
  • biliary cirrhosis due to atresia of bile ducts;
  • cirrhosis in giant cell hepatitis.
• Metabolic disorders:
  • hereditary tyrosinemia;
  • Girke's disease;
  • congenital cystinuria in combination with hemyhypertrophy;
  • insufficiency of a1-antitrypsin.
• Effects of medications:
  • androgens;
  • methotrexate.
• Infectious diseases:
  • chronic viral hepatitis B and C.
• Other diseases and conditions:
  • family adenomatous polyposis.

All patients are evaluated for liver function (determination of transaminase activity, concentration of cholestasis markers, determination of parameters of protein-synthesized function, parameters of blood clotting). Blood is examined for markers of viral hepatitis (primarily B and C).

Stages of liver tumors in children

The classification by stages takes into account the residual volume of the tumor after surgical excision.

• Stage I. Completely distant tumor in the absence of metastases.
• Stage II. Microscopically incompletely removed tumor, absence of metastases; rupture of the tumor during surgery.
• Stage III. Macroscopically incompletely removed tumor or involvement of regional lymph nodes; absence of metastases.
• Stage IV. The presence of distant metastases.

Treatment of liver tumors in children

Surgical removal of a 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 size of the tumor, which contributes to its complete excision. In addition, chemotherapy reduces the risk of intraoperative complications. Adjuvant chemotherapy for hepatoblastoma is performed after complete excision of the tumor in a volume of four courses using cisplatin. Vincristine and doxorubicin. With fully removed hepatocellular carcinoma, the recommendations are of a general nature - repeated courses of chemotherapy with cisplatin and doxorubicin. There are reports of 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 the III-IV stages and with incomplete resection of the tumor, it is possible to use high-dose cisplatin in combination with etoposide. Alternative therapy in these cases is chemoembolization of the tumor through the hepatic artery or orthotopic liver transplantation.

The role of radiation therapy is limited to incomplete tumor removal. As a rule, the effective dose of irradiation exceeds the radiation tolerance of the hepatic tissue. Irradiation, carried out immediately after surgery, significantly slows the regeneration processes in the liver.

Liver transplantation is a highly effective method of treating tumors. Currently, 5-year posttransplantation survival in hepatoblastoma is more than 80%, and for hepatocellular carcinoma - about 65%. Risk factors for the development of relapse in the posttransplant period include tumor size, involvement of lymph nodes, presence of distant metastases, tumor germination into blood vessels, male sex. With hereditary tyrosinemia and familial cholestatic cirrhosis, liver transplantation should be performed as soon as possible before the development of severe organ disturbances and the onset of a tumor.

Treatment of recurrences of hepatoblastoma is successful under condition of their radical removal. The question of the tactics of treatment depends on many factors and is decided individually. With the recurrence of hepatocellular cancer, the prognosis is extremely unfavorable.

Forecast

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

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

To favorable histological variants of liver tumors carry hepatoblastoma with fetal histological structure and fibrolamellar carcinoma; to adverse - hepatoblastoma with embryonic histological structure and hepatocellular carcinoma.