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Hepatoblastoma: causes, symptoms, diagnosis, treatment
Last reviewed: 23.04.2024
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Hepatoblastoma is a rare tumor that affects children under 4 years of age, regardless of sex; it rarely develops in older children and adults.
The first symptoms of hepatoblastoma are a rapid increase in the volume of the abdomen, accompanied by anorexia, a decrease in the child's activity, fever and, in rare cases, jaundice. To manifestations of hepatoblastoma also include accelerated sexual maturation due to tumor secretion of ectopic gonadotropin, cystathionuria, hemihypertrophy and renal adenoma.
The level of a-fetoprotein in the serum is significantly increased. Visualization methods of investigation reveal volumetric education in the liver, displacement from the normal position of adjacent organs, sometimes foci of calcification. On liver angiograms, signs of primary liver cancer can be seen - a diffuse tumor fullness that persists in the venous phase, profuse vascularization of it, foci of contrast agent accumulation and fuzziness of contours.
The histological signs of hepatoblastoma are a reflection of the stages of embryonic liver development, so sometimes teratoid changes are noted. Usually hepatoblastoma is a fetal type tumor with embryonic cells in the acini, pseudo-nodules or papillary structures. Sinusoids contain hematopoietic cells. With a mixed epithelial-mesenchymal type of tumor, a primitive mesenchyme, an osteoid and occasionally cartilaginous tissue, rhabdomyoblasts or epidermoid foci are found.
A relationship between familial adenomatous polyposis of the large intestine and hepatoblastoma has been established. Other combinations are also possible; on chromosome 11, a gene is identified that is associated with hepatoblastoma and other embryonic tumors. Cytogenetic studies have revealed chromosomal abnormalities.
If it is possible to perform liver resection, the prognosis is usually better than with hepatocellular carcinoma; 36% of patients live 5 years or more.
There are also cases of liver transplantation.
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