Comprehensive treatment of hepatoblastoma in children

  The local anatomy of the liver and the compensation of liver function after liver tumor resection are the key issues in liver tumor surgery. Through various imaging examinations before surgery, we can understand the site, extent, and proximity of the tumor, especially the invasion of hepatic vessels. Experienced pediatric hepatobiliary surgeons can often provide a general estimate of whether the tumor can be safely removed in one stage and whether the residual liver can maintain the basic needs of the organism. As a valuable imaging tool, angiography of the liver is important in determining the feasibility of surgery. If hepatic angiography is not possible, the author believes that enhanced CT examination is necessary and very effective. Enhanced CT can show the boundaries of tumor more clearly, especially the relationship between tumor and portal vein and hepatic vein according to the difference of arterial and venous phases, so that the possibility of successful resection can be estimated more accurately before surgery. In recent years, I have successfully resected more than ten cases of huge pediatric liver tumors, the heaviest tumor amounted to 4.8 kg, and another case of 5 months old child with the largest tumor weight accounted for 1/5 of the body weight: 1. Pre-operative preparation In early stage children, the general condition is better, and only simple routine pre-operative preparation can be carried out. However, children with this disease often have poor general condition, malnutrition and hypoproteinemia, etc. They should be given intravenous nutritional support and vitamin K as early as possible.  2.Surgical resection Pediatric hepatoblastoma tumors are often larger, and the proportion of resection is often much larger than that of adults. However, pediatric liver has strong regenerative ability. Some people report that as long as more than 20% of normal liver tissues are preserved, life can be maintained, and the regenerated liver can be restored to its original volume within 2 months, so we should actively strive for complete resection of the tumor.  During surgery, the operation style is selected according to the size and location of the tumor. Tumor resection, lobectomy, hemihepatectomy or enlarged multi-lobe resection of the liver can be performed depending on the situation. For many cases of huge liver tumors, the author first finely dissected the first, third and second hepatic hilum, completely dealt with the related portal vein branches, second and third level hepatic arteries, short hepatic veins, hepatic veins and bile ducts before blocking the first hepatic hilum to start resection of the tumor. More than a dozen cases performed in recent years have had no intraoperative deaths and all have passed the perioperative period unharmed. This surgical method brings new hope to some patients with huge liver tumors that are otherwise inoperable.  In the past, one-sided emphasis was placed on complete surgical resection of the tumor, with no tumor cells found microscopically at the margin of the resected mass. Recently, it is advocated that those who can be safely and completely resected can be completely resected, otherwise only palliative resection of most of the tumor, leaving little tumor tissue, supplemented with chemotherapy after surgery, may survive for a long time.  3.Post-operative treatment After surgery, especially within 2 weeks after surgery, the child must be provided with sufficient nutrition, including the supply of protein, vitamins and energy absolutely needed.  Post-operative chemotherapy, together with comprehensive treatment, is especially important for pediatric liver malignancies. Chemotherapeutic drugs, such as vincristine, cyclophosphamide, and 5-fluorouracil have certain anti-hepatocellular carcinoma effects. Adriamycin is more effective against hepatocellular carcinoma and hepatoblastoma, but has high side effects. Some foreigners reported that 35% of the patients who had been completely resected by visual observation but still had tumor tissue left microscopically survived with postoperative chemotherapy. At present, we advocate the combination of multiple regimens and alternate drug administration. There are also those who undergo hematopoietic stem cell transplantation or bone marrow transplantation.  The treatment of HB with huge tumor (unable to remove the tumor in one stage) Some children with advanced disease often have poor general condition, obvious poor liver function and huge liver tumor, which cannot be removed in one stage surgery. For such children, it is recommended to first open the abdomen for biopsy to clarify the diagnosis. Or for those with very high serum methemoglobin and clear diagnosis, preoperative chemotherapy or interventional therapy with chemotherapy can be performed. After such preoperative treatment, the intrahepatic tumor will shrink significantly, while the normal liver will be relatively enlarged, and more complete tumor resection can be performed.  Pediatric malignant solid tumors have clinical characteristics such as rapid development and early metastasis, and more than half of the children have already metastasized to neighboring tissues, regional lymph nodes, or even distant via blood transport. With the application of preoperative chemotherapy and vascular blockade to control bleeding, the complete resection rate of tumor is nearly 70.0%, among which the complete resection rate of liver malignant tumor reaches 75.0%. Pre-operative and post-operative adjuvant chemotherapy has been widely carried out, which has positive significance to control metastasis dissemination, kill microscopic lesions, preserve limb organs, maintain physiological function and improve survival rate, but some cases cannot adhere to the whole process of chemotherapy and treatment irregularities cannot be ignored.  Liver transplantation for hepatoblastoma (for unresectable HB) Among malignant tumors originating from the liver in children, hepatoblastoma and hepatocellular carcinoma are estimated to be more than 98%. Many tumors are well controlled with preoperative chemotherapy and delayed surgery, and limited tumors are treated with primary tumor resection in one stage. More than 85% of the liver can be safely resected, and the liver can be completely regenerated 3 to 6 months after surgery. Liver transplantation can be performed in cases of unresectable two-lobe multiple liver tumors, vascular invasion, encirclement of the hilum and major ducts, and recurrent liver tumors. Primary and metastatic liver tumors such as hepatoblastoma, epithelioid hemangioendothelioma, hepatocellular carcinoma, and fibrosarcoma are suitable for liver transplantation.  With the advancement of human tissue and organ transplantation, liver transplantation has been gradually applied to the treatment of pediatric hepatoblastoma that cannot be surgically resected. A group reported 5 cases of liver transplantation for unresectable liver tumors, 3 males and 2 females. Two children with hepatoblastoma aged 3 and 6 years old were operated on without extrahepatic metastases. One case had multiple liver tumors and the other case had a right lobe tumor with portal vein embolism, which was confirmed by ultrasound and CT. One case had postoperative hepatic artery embolism, liver abscess, biliary tract obstruction and cholestasis. Both children were healthy and in school by 37 and 25 months after liver transplantation. A 2.9-year-old girl had hepatic hemangioendothelioma, a lesion invading the right and left hepatic lobes and bile ducts. The tumor rapidly enlarged and a total liver transplant was performed. Two children with moderately differentiated hepatocellular carcinoma died 8 and 5 months after transplantation due to recurrence of metastatic tumors.  III. Prognosis and follow-up Clinical staging, pathological type, tumor site, and surgical outcome are the main prognostic factors for pediatric liver treatment. Early detection and early resection remain the key to desirable outcome and long-term survival of hepatoblastoma. The survival rate of children with subclinical stage is significantly higher than that of clinical stage or advanced cases, and the prognosis of highly differentiated types is better. A group of 60 cases of hepatoblastoma in the United States reported survival rates as high as 90% in the subclinical stage and about 30% in stages III and IV. The only way to obtain a good prognosis and long-term survival is to completely resect the hepatoblastoma. In recent years, the application of interventional therapy and preoperative chemotherapy has made it possible for advanced tumors, metastases and recurrent tumors to still have the possibility of complete resection and long-term survival. AFP is an important indicator, and for those with persistently elevated AFP after surgery, ultrasound, CT, chest plain film and isotope whole body bone scan should be actively performed to detect metastases and recurrent lesions. The survival rate of hepatoblastoma is mostly 2 years, but follow-up should be insisted on 5 years.