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Fibrolamellar carcinoma of the liver: causes, symptoms, diagnosis, treatment
Last reviewed: 07.07.2025
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Fibrolamellar liver carcinoma occurs in children and young adults (5-35 years old) regardless of gender.
Initial manifestations of the disease include a palpable tumor in the right hypochondrium, sometimes pain. There is no connection between the tumor development and the intake of sex hormones. There are no cirrhotic changes in the liver.
Histologically, clusters of large polygonal, intensely stained eosinophilic tumor cells are found, interspersed with stripes of mature fibrous tissue. Pale inclusions - deposits of intracellular fibrinogen - can be seen in the cytoplasm of the cells. Sometimes fibrous stroma is absent.
Electron microscopic examination reveals clusters of mitochondria and thick, dense, parallel collagen bands in the cytoplasm. Tumor cells are classified as oncocytes. Excessive amounts of copper-containing protein, presumably produced by tumor cells, are found in hepatocytes.
Serum alpha-fetoprotein levels are normal. Serum calcium levels may be elevated due to pseudohyperparathyroidism. Vitamin B12 binding protein and neurotensin levels may also be elevated.
Ultrasound reveals hyperechoic homogeneous foci. On CT scans, fibrolamellar carcinoma appears as a low-density formation; the intensity of the tumor signal increases significantly with contrast. Calcification may be noted.
In T1-weighted MRI, signals from the tumor and unchanged liver tissue have the same intensity; in T2-weighted images, the signal intensity from the tumor is reduced.
The prognosis for fibrolamellar carcinoma is better than for other forms of liver cancer (life expectancy 32-62 months), although the tumor can metastasize to regional lymph nodes.
Treatment involves liver resection or transplantation.
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