Hepatoblastoma
Contents:
Description:
Gepatoblastoma - a malignant tumor of a liver which develops from embrionalno pluripotential bookmark. Meets at babies more often. The tumor is usually presented by a node of whitish-yellow color which sprouts liver tissue.
The hepatoblastoma occurs at children to 3-year age. It one of the most revealed tumors at chest age. After 5 years this form of a tumor of a liver comes to light very seldom. Average age of detection of hepatocellular cancer makes 12 years.
Hepatoblastoma reasons:
From the risk factors increasing probability of emergence of a hepatoblastoma it should be noted inborn malformations, cirrhosis, polyposes of a large intestine, helminthic invasions, a viral hepatitis In, exchange disturbances, aflatoxins, nitrosamines and some other.
Pathogeny:
Microscopically allocate two types of a hepatoblastoma:
1. epithelial, consisting only of epithelial cells;
2. the mixed, epithelial and mesenchymal type.
In epithelial type find two types of cells: the first look - fetalis, very small (in comparison with normal) hepatocytes with rather large kernel and various number of mitoses.
Trabeculas are located incorrectly, dense ranks, on two young people (as in a fetalis liver) cells in everyone. Also the vacuolated cells filled with lipids and a glycogen meet. Sometimes reveal the hemogenesis centers (what is not present in a normal parenchyma).
The second type of cells - embryonal, small, oblong and dark, located on similarity of sockets. Never neither the centers of a hemogenesis, nor vacuolation, nor lipids and a glycogen are found.
The small-celled undifferentiated component consists of limfotsitopodobny roundish or oval cells with high mitotic activity, ekspreassiruyushchy only CK18 and CK19 cytokeratins.
The mixed type of a hepatoblastoma is usually presented by a combination of fetalis and / or embryonal types of a differentiation of hepatocytes to mesenchyma derivatives (fibrous, ossiform, chondroid fabric) to which the immunoexpression of vimentin, a neyronspetsifichesky enolaza and S100 protein is inherent. Such option of a tumor is designated sometimes as mixed hepatoblastoma oozes without teratoid structures. Less often along with the listed derivatives in a new growth there are islands of cross-striped muscular tissue, a multilayer flat or muciparous epithelium, and also melaninsoderzhashy cells. Such tumors carry to the mixed hepatoblastoma type with teratoid structures.
Hepatoblastoma symptoms:
Special symptoms do not exist. At a palpation of a stomach tumoral weight in right hypochondrium is usually probed or the new growth comes to light in connection with increase in the sizes of a stomach. Anorexia, loss of weight, an indisposition, a febrile state belong to the general signs.
Datas of laboratory: the anemia, a leukocytosis with a polynucleosis and shift to the left raised by SOE, a hyperthrombocytosis in connection with intra tumoral formation of thrombocytes, the increased content of transaminases, lactate dehydrogenases, an alkaline phosphatase and cholesterol (10,3 - 15,5 mmol/l or 400 - 600 mg of %), availability of alpha-fetoprotein in 80% of cases.
Diagnosis:
Usually by the time of clarification of the diagnosis the tumor sizes big, but there are no metastasises yet. The early diagnosis of malignant new growths always makes difficulties in view of a long asymptomatic current.
Stsintigrafiya and ekhografiya allow to define disease topography. The hepatic biopsy is connected with threat of emergence of a hemorrhage. Recommend an early trial laparotomy and, as far as possible, a resection. The hyperthrombocytosis is the instruction on a zlokachestvennost. Availability of alpha-fetoprotein helps clarification of the diagnosis.
The biopsy (capture of a piece of a tumor for microscopic studying) by means of a puncture or during operation is carried out when data of the conducted comprehensive examination are doubtful.
The X-ray analysis of a thorax gives the chance to find metastatic defeat of pulmonary fabric which meets in 20% of cases at the time of diagnosis of the main tumor.
The X-ray analysis of an abdominal cavity allows to reveal calcificats (calcifications) in tumors of a liver and to exclude other tumoral diseases, for example, neuroblastoma metastasises.
Ultrasonography (ultrasonography) helps to confirm a tumor origin from a liver, to specify its arrangement and distribution in body, to reveal defeat of hepatic vessels.
The Computer Tomography (CT) with contrasting, the magnetic and resonant tomography (MRT), radio isotope scanning of a liver, an angiography (a contrast research of vessels) are applied for more information, operation, necessary at planning of volume.
The differential diagnosis needs to be carried out with Vilms's tumor, an adrenal gland neuroblastoma, a neuroblastoma with metastasises to a liver, a pancreas pseudocyst, cystiform expansion of a bilious channel and other tumors in an abdominal cavity.
Treatment of a hepatoblastoma:
Treatment is surgical: a liver resection together with a tumor, chemotherapy or change.
What occurs after the end of treatment?
After end of all program of treatment patients have to be under constant observation of doctors and undergo inspection (a X-ray analysis of a thorax, ultrasonography, KT, MPT).
Important method of a research is determination of the AFP level on the basis of which it is possible to judge efficiency of treatment and emergence of a recurrence (return) of a disease.
The forecast during radical removal of a hepatoblastoma favorable - 60% of patients recover. The forecast at hepatocellular cancer is worse - only 10 - 12% of children recover. At a left-side hemihepatectomy the outcome is better, than at right-hand. Metastasises come to light in lungs, is more rare in a brain, bones.
Prevention:
In view of the fact that the hepatoblastoma has lines of family burdeness (though it is rare), risk of developing of a disease among younger brothers and sisters higher in this connection they should be registered and subjected to periodic control (clinical examination and laboratory researches, in particular on the content of alpha-fetoprotein).
