hepatoblastoma
Introduction
Introduction to hepatoblastoma Hepatoblastoma is the most common liver tumor in children, accounting for approximately 62% of children with primary liver malignancies. The disease originates from liver embryonic primordial cells and belongs to epithelial-derived liver malignant tumors. The disease is highly malignant and can be widely metastasized through blood and lymphatic pathways. The more common metastatic sites are lung, abdominal cavity, lymph nodes and brain. basic knowledge The proportion of illness: 0.001% Susceptible people: good for children Mode of infection: non-infectious Complications: cleft palate, giant tongue, umbilical hernia
Cause
Hepatoblastoma etiology
Cause of the disease (75%):
The cause may be abnormal development of embryonic connective tissue, which is a malignant tumor that occurs in the liver primordial cells. Newborns may occur in the uterus. It has been reported in the fetus at 7 months of pregnancy. Adults may be embryonic hepatocytes. Malignant tumors occur only after many years in the liver.
Pathogenesis (15%):
Hepatoblastoma mostly occurs in the right lobe of the liver. About half of the cases invade the two leaves or are multi-centered. Generally, they are single-round circular boundaries. The diameter of the tumor ranges from 5 to 25 cm. The general appearance of the tumor is related to the mesenchymal tissue components such as bone-like cartilage or fibrous tissue. Generally speaking, the tumor is rough nodular or lobulated in the liver surface, the boundary is clear, half of the capsule is covered, and the texture is hard. The color of the tumor is brown to gray. The texture of the section is uniform and uniform, and bleeding, necrosis and calcification may occur. The cirrhosis microscope shows that the tumor consists of epithelial components and interstitial variability components at different growth stages of maturity. It can be divided into fetal (highly differentiated) embryonic (lowly differentiated) and mixed types according to the degree of differentiation of tumor cells.
1. Fetal type: fetal liver cells are smaller than normal liver cells in a polygonal cell boundary. Clear cytoplasm is eosinophilic granules, and there may be a ratio of vacuolar cells to nucleoplasm of 1:4 to 1:2. The nucleus is round or oval. Chromatin varies in size, and a single nucleoli nucleus is rare. The cells are arranged in two cell thick irregular liver cells between the liver bundles and the liver sinus. Analysis of nuclear DNA content in hepatoblastoma cells showed that the fetal type was mostly diploid.
2. Embryonic type: embryonic liver epithelial cells differentiate poor cell small spindle cell boundary fuzzy cytoplasmic nucleoplasm ratio of 1:1 to 2, nuclear chromatin is rich, nucleoli large and obvious mitotic figures are easy to see. This type of cell adhesion ability is poor, often clustered into rose petal-like or connected to loose cord-like hepatoblastoma cell nuclear DNA content analysis shows that embryonic type is mostly aneuploid aneuploid tumor is prone to vascular invasion, poor prognosis is common The extrahepatic metastasis is the lungs, abdominal lymph nodes and brain.
3. Mixed type: Mixed hepatoblastoma also contains other interstitial components such as bone-like tissue cartilage, fine-striped muscles, squamous cell foci, and keratinized beads.
Prevention
Hepatoblastoma prevention
The cause is still unknown. Refer to the general tumor prevention methods, understand the risk factors of tumors, and formulate corresponding prevention and treatment strategies to reduce the risk of tumors. There are two basic clues to prevent tumors. Even if tumors have begun to form in the body, they can help. The body improves resistance, and these strategies include:
1. Avoid harmful substances (promoting factors) that can help us avoid or minimize exposure to harmful substances.
2. Improve the body's immunity against tumors can help improve and strengthen the body's immune system and cancer.
Complication
Hepatoblastoma complications Complications
Occasionally, huge tumors rupture, causing symptoms and signs of acute abdomen, and a small number of children with congenital malformations, such as cleft palate, giant tongue, auricular dysplasia, lack of right adrenal gland, umbilical hernia, cardiovascular malformation or kidney Malformation and so on.
Symptom
Hepatoblastoma symptoms Common symptoms Loss of appetite, diarrhea, ascites, pale, anorexia, liver sag
Clinical symptoms are non-specific, often have loss of appetite, anorexia, weight loss or no increase, upper abdominal pain accompanied by vomiting and / or diarrhea, jaundice is rare, abdominal bulging is a common symptom of children with hepatoblastoma, children Parents often notice or pass the physical examination to find the upper abdomen mass, and most of the sick children see a doctor because of abdominal enlargement.
Signs are mainly pale, abdominal swelling, liver enlargement, physical examination in the right upper abdomen can touch the swollen liver, hard texture, smooth surface, the larger can reach the pelvic cavity, can touch the tumor nodules with tenderness, late can There are jaundice, ascites, and other symptoms include dysplasia, irritability and so on.
Some children with hepatoblastoma have precocious puberty as the initial symptom, the incidence rate is 2.3%, which is manifested as genital enlargement, low tone and pubic hair growth. The reason is that tumor cells can synthesize human chorionic gonadotropin (HCG). .
Examine
Hepatoblastoma examination
1. General laboratory inspection
There may be a decrease in hemoglobin, a decrease in red blood cells and platelets; a mild abnormality in liver function, a mildly moderate increase in AKP and IDH; AFP may be significantly elevated in most patients, AFP is 80% to 90% positive, and 100% is elevated. Can be up to 100,000 ng / mL; about 50% of children with urinary excretion of cystathionine, male precocious children with serum and urine human chorionic gonadotropin (HCG), luteinizing hormone and testosterone levels.
2. Biopsy
Percutaneous liver biopsy can confirm the diagnosis.
Imaging studies can detect large space-occupying lesions in the liver and are valuable for the diagnosis of hepatoblastoma.
3.B Ultra
Ultrasound showed an isolated mass with enhanced heterogeneous echo, and the liver partially increased and lost its normal shape. The liver showed a massive strong echo, the internal strength was different, the distribution was uneven, and there was a mixed echo image when liquefied, occasionally It can be found that there are punctate or irregular calcifications in the tumor, and the tumor envelope is intact.
4. CT examination
CT showed low-density massive tumors with reduced CT values, but it was difficult to identify with multiple blood donors and liver cancer. The performance was as follows:
(1) Sweeping single or multiple low-density masses in the liver parenchyma, homogeneous or heterogeneous (with more heterogeneity), the boundary is clear or unclear, and the enhancement of scanning lesion heterogeneity is enhanced, mainly around the lesion.
(2) Most of the tumors are large, and the liver is often enlarged and deformed. The masses are massive or massive and multi-nodular.
(3) Tumors often involve one leaf of the liver, and may also involve two leaves or multiple centers.
(4) About half of the cases showed calcification in the lesion.
5. MRI examination
MRI showed a weak and weak signal zone, and hepatic angiography had a localization effect on hepatoblastoma and suggested whether the surgery could be removed.
6. Other
In addition, angiography and radionuclide scanning can be performed.
Diagnosis
Diagnosis and identification of hepatoblastoma
diagnosis
According to the young age of the patients, some may have abdominal distension and anorexia soon after birth, abdominal distension, weight loss, abdominal (including abdominal wall, abdominal cavity and retroperitoneal space) mass can be physiological, such as filled bladder, pregnant uterus Dry stools, etc.; more important is pathological, is the clinical manifestation of some abdominal diseases. The so-called abdominal mass refers to more or associated precocious puberty. AFP-enhanced imaging confirmed that intrahepatic space-occupying mass combined with auxiliary examination can make a diagnostic biopsy to confirm the diagnosis.
Differential diagnosis
The disease must be differentiated from primary liver cancer, hepatic hemangioma, hamartoma and teratoma, and differentiated from extrahepatic lesions such as retroperitoneal Wilms tumors and neuroblastoma, especially hepatocellular carcinoma, but The latter is more common in adults and those with a history of hepatitis. They often have large liver, liver pain is associated with cirrhosis, AFP is positive, and the content is increased. At the same time, ALP, GGT and LDH are also increased.
Hepatic cyst
(1) The course of disease is long and the condition progresses slowly.
(2) often no liver disease background.
(3) The general situation is good.
(4) Ultrasound examination showed cystic structure and fluid level.
2. Hepatic hemangioma
(1) The course of disease is long and progress is slow.
(2) There is often no history of chronic liver disease.
(3) The general situation is good.
(4) Women are more common.
(5) 99mTc-RBC radionuclide scanning is a "hot" zone.
(6) The imaging examination has no capsule, and the contrast agent is injected and enhanced from the periphery.
(7) Liver function and enzyme, normal spectrum examination.
