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Fibrolamellar carcinoma of the liver: causes, symptoms, diagnosis, treatment
Last reviewed: 23.04.2024
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Fibrolamellar carcinoma of the liver occurs in children and young people (5-35 years) regardless of sex.
The initial manifestations of the disease include a palpable tumor in the right upper quadrant, sometimes pain. There is no association of tumor development with the reception of sex hormones. Cirrhotic changes in the liver are absent.
Histologically, clusters of large polygonal, intensely colored eosinophilic tumor cells interspersed with bands of mature fibrous tissue are found. In the cytoplasm of cells, pale inclusions can be seen - the deposits of intracellular fibrinogen. Sometimes the fibrous stroma is absent.
Electron-microscopic examination reveals clusters of mitochondria and thick dense, parallel strips of collagen in the cytoplasm. Tumor cells are referred to oncocytes. In hepatocytes, an excessive amount of copper-containing protein is detected, which is produced presumably by tumor cells.
The serum a-fetoprotein level is normal. Due to pseudo-hyperparathyroidism, the serum calcium level can be increased. It is also possible to increase the level of protein that binds vitamin B12, and neurotensin.
When ultrasound is detected, hyperechoic homogeneous foci. On computer tomograms, fibrolamellar carcinoma has the appearance of a reduced density; the intensity of the signal from the tumor increases significantly when contrasting. Calcification can be noted.
With MRI in T1-mode, signals from the tumor and unchanged liver tissue have the same intensity, on T2-weighted images, the intensity of the signal from the tumor is reduced.
The prognosis for fibrolamellar carcinoma is better than with other forms of liver cancer (life expectancy 32-62 months), although the tumor can metastasize to regional lymph nodes.
Treatment consists of resection or liver transplantation.
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