Despite the fact that the liver tumor in children is a rare phenomenon, nevertheless, 70% of them are among malignant tumors. In this series, hepatoblastomas occupy a leading position - they account for 80% of cases of primary malignant neoplasms of the liver.
Concept of illness and statistics
According to foreign medical statistics, among the population of Europe and North America , the share of primary liver cancer accounts for slightly more than 1% of childhood and 10% of all other cancers .
Developing most often in the tissues of the right lobe of the liver, hepatoblastoma is a dense, capsule-free growth( it can be either single or multiple) of a yellow and white color that resembles a node and is capable of producing bile.
Free-germinating in the tissue of the affected organ, hepatoblastoma differs pronounced lobular structure and the presence of foci of necrosis and hemorrhages.
Hepatoblastoma tissues contain a large number of immature and different hepatocytes, which are completely unsuitable for the performance of their inherent functions due to the lack of development of these cells.
The photo shows hepatoblastoma of the liver
Microscopic analysis of the tissues that make up the basis of the hepatoblast allows them to be divided into two morphological types:
Causes of a malignant tumor
Any exact reasons provoking the development of hepatoblastoma, modern medicine are still unknown. Medical statistics state that hepatoblastoma is often detected in children suffering from hereditary anomalies endowed with malignancy.
Such anomalies include:
- Wiedemann-Beckwith syndrome;
- hemygypertrophy( facial and body asymmetry);
- family adenomatous polyposis;
- hereditary tyrosinemia( a disease characterized by the defeat of three vital organs: the pancreas, liver and kidneys).
There are frequent cases of a combination of hepatoblast with nephroblastomas( malignant tumors of the kidneys).Sometimes an impetus to the development of a malignant liver tumor is hepatitis B, which is carried by a baby, as well as the infection of his body with eggs of helminths( helminthic invasion).
There is an assumption that there is a relationship between the likelihood of hepatoblastoma and the intake of some oral contraceptives by a future mother( even before pregnancy).
The severe alcoholic syndrome of the fetus and the presence of glycogen disease of the first type are among the serious risk factors.
Symptoms of hepatoblastoma
For hepatoblastoma, the initial period of development is characterized by asymptomatic leakage. The first manifestations and the characteristic clinical picture appear when the disease, progressing, reaches the stage of metastasis.
The number of specific manifestations of hepatoblastoma includes the presence of:
- increased in volume, bulging belly;
- of the bulk seal localized in the right hypochondrium region;
- of severe pain syndrome.
Further progression of the malignant liver tumor leads to loss of appetite, some weight loss, increased weakness, general malaise and mild nausea. The presence of dyspeptic disorders, manifested in the development of heartburn, belching, vomiting, stool disorders( in the form of constipation or diarrhea) with hepatoblastoma is extremely rare.
The addition of vomiting, jaundice of the skin, a sharp decrease in body weight, a persistent increase in body temperature to the level of febrile values is evidence that the tumor process has reached its final stage.
In very rare cases, when tumor tissues produce a specific hormone substance - chorionic gonadotropin( called pregnancy hormone), a child may experience early puberty.
Diagnosis of the liver in children
Methods of hepatoblast diagnosis are not specific. Given the asymptomatic course of the disease, it is very rare to detect hepatoblastoma at its early stages.
In the overwhelming majority of cases, the detected tumor is already large, but fortunately for the patient, there are still no metastases.
In modern clinics, a full pathological examination is carried out, which places special emphasis on the analysis of liver parameters.
- When a child is physically examined, the specialist can easily determine the presence of a seal located in the right hypochondrium.
- Because liver biopsy is fraught with the development of severe hemorrhage( hemorrhage), doctors prefer to resort to an early trial laparotomy( an operation that opens access to internal organs through a microcut of the abdominal wall).During the revision of the abdominal cavity for laboratory analysis, samples of tumor tissue and lymph nodes located close to the hepatoblastoma are taken. Sometimes in the course of diagnostic laparotomy, a specialist can perform a complete resection( removal) of a malignant neoplasm.
- Ultrasound examination of the liver and abdominal organs allows to reveal the localization of the tumor, the degree of its spread, the number of seals( nodes), the presence of lesions that touched the large blood vessels of the liver.
- To clarify the topography of the disease, echography and scintigraphy procedures help.
- For chest metastases, chest radiography is used. According to statistics, at the time of diagnosis of the tumor, they are noted in every fifth patient.
- Radiography of the abdominal cavity is performed to identify calcifications in the hepatic neoplasms, allowing to exclude the presence of other tumor processes.
- When developing a plan for a future operation, a specialist assigns a whole range of studies: computed tomography, angiography, liver tissue scanning( radioisotope), magnetic resonance imaging. The data from these studies help to clarify the amount of surgical intervention required.
- With certain doubts about the correctness of the diagnosis, a biopsy of tumor tissue is performed. A small sample is taken during surgery or by performing a puncture.
- At the stage of differential diagnosis hepatoblastoma is separated from a number of diseases with similar symptoms( Wilms' tumors, pancreatic pseudocysts, cystic bile duct enlargement, etc.).
Treatment of hepatoblastoma is radical and involves the only way - surgical resection of a malignant neoplasm, performed simultaneously with liver fractions( one or more).
The high regenerative capacity( regenerative potential) of this organ allows it to restore its original dimensions after a certain time.
The operation, during which segmental fracture resection is performed, is called a lobectomy. Surgery aimed at removing one of the liver halves is called hemihepatectomy. Depending on which half is resected, the operation is left-sided or right-sided.
In particularly difficult cases, surgery is preceded by a course of chemotherapy. Treatment with medications reduces the size of the malignant neoplasm and thereby reduces the scope of future surgical intervention.
Hepatoblastoma, contracting in size, lends itself more completely to excision. Even if the operation was successful, small patients are prescribed a course of postoperative chemotherapy. Its purpose is to cope with cancer cells that were not removed during the operation.
Other treatments for
Radiation therapy is used as a palliative half-measure that alleviates the suffering of operated patients. With its help, specialists manage to stop pain syndrome and reduce manifestations of jaundice.
With late diagnosis of hepatoblastoma, which managed to give metastases, the baby is prescribed a long course of antibiotic treatment and chemotherapy. Sometimes it is expedient to transplant a healthy organ( transplantation) of the liver.
Classification of malignant liver tumors by stages is performed, taking into account the residual volume of neoplasm after resection of the affected organ.
- Hepatoblastoma, completely removed during surgery and not metastasized, corresponds to the first stage.
- The second stage is assigned to hepatoblastoma, which did not manage to give metastases, but did not give in to complete removal during surgery( a part of cancer cells remained in the affected organ).
- The third stage corresponds to a tumor that is not completely removed at the macroscopic level and involves regional lymph nodes in the pathological process. There are also no metastases at this stage.
- The fourth stage is attributed to a disease that caused distant metastases.
The prognosis for hepatoblastoma of the liver is directly dependent on the time of its detection and on a whole range of factors: the age and sex of the patient, the tolerance of his organism to the prescribed treatment, the stage of hepatoblastoma at the time of diagnosis and even the effectiveness of the co-operation of the treating specialists.
A child who has undergone a course of treatment is placed under constant supervision of a number of specialists: a gastroenterologist, an oncologist and a hepatologist who will periodically direct him for the delivery of specific tests( for example, blood tests for alpha-fetoprotein AFP), as well as chest radiographyand ultrasound examination of the abdominal cavity and liver.