Pediatric Liver Tumors

Introduction to Pediatric Liver Tumors As with adults, secondary liver tumors in children are more common than primary liver tumors. Primary liver malignancies account for 1.2% to 5% of pediatric tumors, the most common of which are hepatoblastoma and hepatocellular carcinoma. Benign tumors are mainly hemangioma, hamartoma and teratoma. . Hepatoblastoma is more common than hepatocellular carcinoma, almost all occur in children before the age of 5, and about 50% of patients are younger than 18 months. Boys are more common than girls, especially hepatocellular carcinoma, male: female is 2:1. The peak age of hepatocellular carcinoma is 10 to 14 years old. Similar to nephroblastoma, congenital malformations such as hemifacial hypertrophy and extensive hemangioma can be complicated by hepatic malignancies. Liver tumors and nephroblastoma can occur in the same patient. It has been reported that hepatoblastoma and hepatocellular carcinoma occur in twins. basic knowledge The proportion of illness: 0.001% Susceptible people: children Mode of infection: non-infectious Complications: jaundice, ascites, osteoporosis, anemia

Causes of liver tumor in children

(1) Causes of the disease

The cause of liver cancer, after many studies, although there is a certain understanding, but its reasons have not been known so far, the current research believes that the cause of the disease:

1. Liver cirrhosis Hepatic liver cancer patients with about 80% of patients with hepatitis cirrhosis, and most patients with large nodular cirrhosis, which may be due to hepatocyte degeneration and necrosis, interstitial connective tissue hyperplasia, fibrosis formation, residual hepatocytes Nodular regeneration, formation of lobules, hepatocytes can be mutated during repeated hyperplasia and eventually lead to cancer.

2. Viral hepatitis Hepatitis B is closely related to liver cancer. In HbsAg-positive patients, the incidence of liver cancer is significantly higher than that of HbsAg-negative disease. Hepatitis C is also closely related to the occurrence of liver cancer.

3. After the food containing aflatoxin is ingested, it is absorbed through the digestive tract and reaches the liver, causing degeneration and necrosis of the liver cells, which in turn proliferate and become cancerous.

4. Chemical carcinogens are now known as nitrates and nitrites.

5. The immune status of the body The occurrence of liver cancer is generally associated with low immune function of antibodies, especially in relation to low cellular immune function.

(two) pathogenesis

Hepatoblastoma is mostly a single lesion located in the right lobe of the liver with or without an envelope. The cut surface is gray to brown, with bleeding, ossification and necrosis. In 1967, Ishak and Gkunz divided hepatoblastoma into two types: Epithelial and epithelial-mesenchymal, pure epithelial tumors are nodular, uniform and uniform, consisting of two types of cells, one of which is a fetal cell, shaped like fetal liver cells, often arranged in two cell-thick irregularities Liver plate, different in cell size, but often smaller than normal hepatocytes, cytoplasmic eosinophilic, glycogen-containing, nuclear round or oval, basophilic, with a few mitotic figures, and secondly embryonic cells, slightly differentiated Poor, arranged in bundles, small cells with deep staining, less cytoplasm, little or no glycogen, deep nuclear staining, common mitotic figures, mixed tumor sections separated by collagen fibers and lobulated, visible fetal cells And embryonic cell distribution, supported by reticular fibers, primitive mesenchymal cells are long fusiform, cytoplasm is less, bone-like tissue is visible, in recent years, CCSG (Children's Cancer Study Group), SWOG (Southwest Oncology Group) and POG ( Paediatric Oncology Group) According to the recommendations of Weinber and Finnegold, hepatoblastoma is divided into fetal type, embryo type, thick beam type and small cell undifferentiated type (inter-variant type). Hepatocellular carcinoma contains well-differentiated large-angle cells with eosinophilic cytoplasm, cells. The structure of the hepatic cord is formed, surrounded by vascular sinus. Both tumors can be seen with extramedullary erythrocyte hyperplasia, and more often the right lobe of the liver. About half of the patients invade the right and left lobe or are multicentric, most often transferred to It is not uncommon for the lungs to spread locally in the abdominal cavity. It is less common to transfer to the central nervous system.

Pediatric liver tumor prevention

Pediatric hepatocellular carcinoma often occurs in cirrhosis or original hepatic parenchymal lesions. For example, cases of hepatic malignancies secondary to cirrhosis of the biliary atresia and megacytic hepatitis are increased, and active prevention and treatment should be carried out.

Pediatric liver tumor complications Complications jaundice ascites osteoporosis anemia

Often complicated by jaundice, ascites, osteoporosis, can lead to multiple fractures, anemia and thrombocytosis.

Pediatric liver tumor symptoms Common symptoms Alpha-fetoprotein increased ascites jaundice hepatomegaly hyperthermia heart failure osteoporosis dyspnea liver vascular murmur thrombocytosis

Most children with irregular localized hepatomegaly as the initial symptoms, the mass is located in the right abdomen or right upper abdomen. It is often recorded in the history that the tumor grows rapidly, some can reach the umbilicus or beyond the midline, the surface is smooth, the edge is clear, the hardness is medium, slightly Move left and right, no tenderness, in addition to mild anemia in the early stage, the general condition is good, in the late stage, jaundice, ascites, fever, anemia, weight loss, venous engorgement can be seen in the abdominal wall, and breathing difficulties can be caused by a huge mass in the abdomen. About 20% of cases of hepatoblastoma have osteoporosis, and severe cases can lead to multiple fractures.

Many patients have anemia and thrombocytosis at the time of treatment, especially in children with hepatoblastoma. The liver function of children with hepatoblastoma is normal, but cases of hepatocellular carcinoma are complicated by hepatitis or cirrhosis, serum bilirubin. Alkaline phosphatase and transaminase may increase, alpha-fetoprotein increased in 60% to 90% of hepatocellular carcinoma cases and more than 90% of hepatoblastoma cases, and intravesical cysteine (cystathionine) excretion increased in hepatoblastoma.

Other primary liver tumors are common cavernous hemangioma and vascular endothelial cell tumor. The former can compress liver tissue due to tumor growth, degeneration of liver cells, and sometimes arteriovenous short circuit in tumor, which can cause heart failure or cause in children. Tumor rupture and death, small hemangioma grows slowly, and there is no clinical symptoms.

Vascular endothelial cell tumors are malignant, with sinusoid formation in the liver. There may be pain in the clinic, high fever and jaundice, and the course is slow, but the prognosis is poor.

Hepatic hamartoma, teratoma and single or multiple hepatic cysts are rare.

Pediatric liver tumor examination

The continuous development of serology and imaging provides various methods for the early diagnosis of subclinical liver cancer. Clinically, serological diagnosis is called "qualitative diagnosis", imaging diagnosis is called "positioning diagnosis", needle biopsy or exfoliated cell examination. A comprehensive application of these methods called "pathological diagnosis" can improve the accuracy of the diagnosis.

1. Alpha-fetoprotein (AFP) detects AFP on liver cells with an accuracy of about 90%, and its clinical value is:

(1) Early diagnosis: It can diagnose subclinical lesions, and can make a diagnosis about 8 months before symptoms appear.

(2) differential diagnosis: because 89% of patients with hepatocellular carcinoma have AFP greater than 20ng/ml, so alpha-fetoprotein is lower than this value and there is no evidence of other liver cancer, which can rule out liver cancer.

(3) It helps to reflect the improvement and deterioration of the condition. The rise of AFP indicates deterioration, and the decline is improved if the clinical condition is improved.

(4) It is helpful to judge the thoroughness of the surgical resection and predict the recurrence. If the AFP drops to the normal value after the operation, the resection is complete, and the resuscitation prompts the recurrence. It can also be done 6 to 12 months before the recurrence symptoms appear. Forecast.

(5) It is helpful to evaluate various treatment methods. The higher the AFP conversion rate after treatment, the better the effect.

AFP false positives, not all AFP-positive patients have liver cancer, AFP false positives are mainly seen in hepatitis, cirrhosis, which account for 80% of false positive cases, in addition to gonad embryo cancer, digestive tract cancer, pathological pregnancy, Hepatic vascular endothelium, malignant hepatic fibrosis, etc., diagnosis of AFP-negative patients, AFP-negative can not exclude the diagnosis of liver cancer, enzymology can be performed, among which the clinical significance is: 1 anti-trypsin (AAT), - Glutamate transferase (-GT), carcinoembryonic antigen (CEA), alkaline phosphatase (AKP), etc., these serological test results may rise in patients with liver disease, but not specific.

2. Liver biopsy can not perform liver puncture examination for the diagnosis. Because liver puncture has certain complications, the most common is hemorrhage. In addition, the needle will pass through the portal vein or hepatic vein and biliary tract during liver puncture. In this case, cancer cells may be carried into the blood vessels, causing metastasis.

3. B-ultrasound can show tumors larger than 1cm, the diagnostic accuracy rate is 90%, can show tumor size, location morphology, number, hepatobiliary duct, portal vein, spleen, abdominal lymph nodes, etc., at the same time for cirrhosis, splenomegaly A diagnosis can be made.

4. CT has a diagnostic accuracy rate of 93% for liver cancer and a minimum resolution of 1.5 cm. The advantage is that it can directly observe the size and location of the tumor and the relationship between the hepatic vein and portal vein, and can diagnose the tumor or thrombus in the portal vein or hepatic vein.

5. Angiography Hepatic angiography can understand the blood flow of the lesion to determine the possibility and indication of the operation. It can show tumors of about 1.5cm. It is the highest resolution in the current imaging diagnosis method. Hepatic hemangioma has important significance. It can also understand the variation of hepatic artery at the same time of clear diagnosis. It is very important for hepatectomy. If it is advanced liver cancer, it can be embolized and can not be treated. (or) chemotherapy.

6. MRI and CT are basically the same, but for some difficult to identify liver mass, T1, T2 images can clearly distinguish liver cancer, hepatic hemangioma, liver abscess, cyst and so on.

7. Radionuclide scanning can be identified by blood flow scanning in patients who are difficult to distinguish from hemangioma. Because of the low resolution of radiological scanning, it is rarely used as a diagnostic method for liver cancer.

8. Laparoscopy in patients with difficult diagnosis can be considered laparoscopic examination, direct observation of the liver, liver surface mass and abdominal cavity.

9. X-ray examination showed that right sacral elevation, movement limitation or limited bulge under X-ray fluoroscopy, 30% of cases showed calcification in the X-ray film, and about 10% of cases had lung metastases at diagnosis.

Diagnosis and diagnosis of liver tumor in children

diagnosis

According to the clinical manifestations and the laboratory, the results of the auxiliary examination can be judged, the main points of clinical diagnosis:

1. History of past hepatitis.

2. Liver pain and other clinical symptoms.

3. AFP>400 g/L for more than 4 weeks, or AFP>200 g/L for more than 8 weeks.

4. B-ultrasound, CT found that the liver has a space-occupying lesion, which can exclude metastasis or benign tumors.

Differential diagnosis

The primary difference between the cause of liver disease, whether it is a metabolic disease, benign liver tumors, such as hepatic cavernous hemangioma and liver metastases such as neuroblastoma, by alpha-fetoprotein determination, ultrasound detection and CT or nuclear magnetic can help diagnose.

Intravenous pyelography can be distinguished from retroperitoneal tumors such as nephroblastoma, neuroblastoma, and teratoma.

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