Hepatic angiosarcoma
Introduction
Introduction to hepatic angiosarcoma Angiosarcoma (angiosarcoma), also known as hepatic malignant hemangioma (malignanthemangiomaofliver), hepatic vascular sarcoma, Kupffer cell sarcoma, vascular endothelial cell sarcoma or malignant hemangioendothelioma, is the most common type of liver sarcoma in western developed countries. It is a primary malignant tumor formed by the abnormal growth of hepatic sinusoid cells. basic knowledge The proportion of illness: 0.002% Susceptible people: no specific population Mode of infection: non-infectious Complications: cirrhosis, congestive heart failure, disseminated intravascular coagulation, hemolytic anemia
Cause
Hepatic angiosarcoma etiology
(1) Causes of the disease
Hepatic angiosarcoma is an interstitial tumor with a high degree of malignancy. The etiology of most patients is unknown. Folk et al investigated 168 cases, of which 75% were unexplained. In recent years, some hepatic angiosarcoma related to chemical substances has been reported abroad. People are concerned that it is related to exposure to vinyl chloride. Seven of the 1,183 workers in a vinyl chloride plant in the United States have hepatic angiosarcoma with an average incubation period of 17 years. A few can also occur after taking synthetic steroids, estrogen and contraceptives. The incubation period is more than 10 years.
Vinyl chloride is a gas produced by the process of polyethylene production. Chloroethylene is converted into mutagenic and carcinogenic metabolites by the action of endoplasmic reticulum enzymes, and then covalently bound to DNA. Experiments show that rats or humans can be exposed to vinyl chloride for a long time. Inducing hepatic angiosarcoma, drinking water containing inorganic arsenic, pesticides such as inorganic arsenic and copper sulfate in the vineyard, hepatic angiosarcoma may occur in long-term exposure, Hiroshima atomic bomb explosion and external exposure to radium infusion for 36 years and 3 years respectively There were 1 case of angiosarcoma, which was also found in patients with prolonged application of potassium arsenite for the treatment of psoriasis. Other substances causing hepatic angiosarcoma were radium, inorganic copper and monoamine oxidase inhibitors, and angiosarcoma with idiopathic hemochromatosis. Reported factors related to the onset of human hepatic angiosarcoma (Table 1).
(two ) pathophysiology
General examination
Angiosarcoma often occurs in a multicentric manner, involving two hepatic lobe. Its macroscopic features are many blood-filled sacs with a diameter of 1 to 15 cm. It is more common in 5 cm, and the tumor is mostly a single circle. The surface is multi-nodular, with clear boundaries, but no capsule. The larger mass is spongy. The maximum weight is 3kg, which may be accompanied by thrombosis and secondary necrosis. The cut surface is gray or brownish yellow jelly. The swelled tissue and the hemorrhage and the necrotic foci are staggered. The large cavity filled with non-clotting fluid can be seen in the tumor. In the case of contact with colloidal cerium oxide or vinyl chloride, reticular fibrosis is common, and the spleen is swollen. However, patients with cirrhosis are rare.
2. Histopathological examination
The tumor is composed of spindle-shaped or irregularly shaped malignant endothelial cells. The boundary is unclear, the cytoplasm is eosinophilic, the nucleus is deeply stained, the shape is narrow or irregular, the nucleus nucleus can be large or small, and the same eosinophilic is also visible. Heteromorphic nuclei and multinucleated cells, mitotic figures are common, thin-walled veins are scattered in tumors, and hematopoietic cells are seen in about half of the cases. Immunohistochemical examination shows that there are VIII-factor-related antigens in the tumor cells, and the tumor cells are along the original blood vessels. Cavity proliferation, liver terminal venules and portal vein branches also show proliferation, tumor cells proliferating in the hepatic sinusoids can lead to liver cell atrophy, liver plate rupture, vascular lumen enlargement to form cavities of different sizes, rough wall The inner wall is lined with tumor tissue cells, sometimes polypoid or papillary protrusions. The cavity is filled with clots and tumor fragments. Tumor invasion into the terminal venules and portal veins of the liver can cause blockage of these blood vessels, which explains why the tumors It is often prone to bleeding, infarction and necrosis, and sometimes the tumor cells are densely clustered into small nodular solid masses, similar to fibrosarcoma.
Immunohistochemical techniques can be used to detect positive factor VIII-associated antigen in tumor cells.
In most cases, the tumor can invade the portal vein or central vein. This happens mostly in the tumor nodules, but it can also occur in distant hepatic lobules. Extramedullary hematopoietic foci are common and can be secondary to microvascular hemolytic anemia. Iron deposition, in the hepatic angiosarcoma induced by colloidal cerium oxide, it is easy to see glial cerium dioxide deposited in the reticuloendothelial cells or in the portal area, Glisson sheath or hepatic terminal venule wall, The sediment is colorless and can be refraction. HE staining deposits are usually pink and brownish yellow. Autoradiography can show rays emitted by cerium oxide, which is a short point trajectory. Energy dispersive X-ray microanalysis can also be measured. Anthraquinone, angiosarcoma caused by cerium oxide, arsenic or vinyl chloride, cirrhosis and fibrosis in non-tumor tissues, about one-third of patients with idiopathic cirrhosis, tumors caused by clear pathogenic factors There is no morphological difference between it and idiopathic tumors.
Prevention
Hepatic angiosarcoma prevention
Since the occurrence of this disease is related to exposure to harmful chemical substances and radioactive substances, it should be avoided, and occupational workers should do their own protection.
Complication
Hepatic angiosarcoma complications Complications, cirrhosis, congestive heart failure, disseminated intravascular coagulation hemolytic anemia
About 25% of the disease with cirrhosis, tumor rupture can cause bloody abdomen, hepatic angiosarcoma is easy to be complicated by intra-abdominal and gastrointestinal bleeding, due to the presence of shunt in the tumor can be complicated by congestive heart failure, hepatic angiosarcoma patients due to platelets in the tumor Accumulation often has thrombocytopenia and abnormal blood coagulation. Platelets are consumed in large amounts in the tumor, which can cause disseminated intravascular coagulation with secondary fibrinolysis and microvascular hemolytic anemia caused by rupture of red blood cells in the tumor blood circulation.
Symptom
Hepatic angiosarcoma symptoms Common symptoms Abnormal liver function, abdominal pain, jaundice, abdominal discomfort, nausea, ascites, loss of appetite, acute abdomen, weight loss
The main symptoms at the beginning are abdominal pain or discomfort. Other common complaints include bloating, rapid progression of liver failure, weakness, loss of appetite, weight loss, and the following ways:
1. More than half of the liver is unexplained, accompanied by some gastrointestinal symptoms, such as liver enlargement, abdominal pain, abdominal discomfort, fatigue, nausea, poor appetite, weight loss, occasional vomiting and fever, etc. In the advanced stage, there may be jaundice, ascites, and ascites.
2. Tumor rupture causes symptoms and signs of acute abdomen caused by bloody abdomen.
3. A small number of patients may have splenomegaly with or without complete cytopenia.
4. Often there are extrahepatic metastases, mostly blood-borne, may have signs and signs of metastasis to the lungs, pancreas, spleen, kidney and adrenal gland or bones, with lung metastases being the most common, symptoms lasting from 1 week to 6 months, For 2 years.
The liver is swollen and has nodules on the surface. In some patients, the surface of the liver can touch the mass. Most of them have tenderness. The surface of the liver can sometimes smell arterial murmurs. The spleen can be swollen. Ascites is rare, but it can be bloody ascites. Patients are often accompanied by jaundice.
Clinical symptoms and signs are non-specific, have a history of exposure to vinyl chloride, often have white blood cells, thrombocytopenia, prolonged prothrombin time, abnormal liver function, elevated ALP, hyperbilirubinemia, X-ray, CT, liver nucleus Scans found variations in liver mass and filling defects, and Burston proposed three diagnostic criteria for reference:
1 pathological morphology like Kupffer cells.
2 has a tendency to form blood vessels.
3 There is phagocytosis, and the diagnosis is most reliable with liver biopsy.
Examine
Hepatic angiosarcoma examination
1. Blood examination: may have anemia, microvascular hemolytic anemia, leukocytosis (65%) or leukopenia (25%), thrombocytopenia (62%).
2. Liver function tests: About 2/3 of patients have abnormal liver function: 100% of patients with positive sulfonium bromide retention test (BSP), 85% of ALP, and 60% of hyperbilirubinemia Some patients have elevated ALT.
Approximately 50% of patients have a mild to moderate elevation of transaminase.
3. Diffuse intravascular coagulation: a rare complication of the disease, prolonged prothrombin time accounted for 72%.
4. p53 gene: Smith reported that the mutation of p53 tumor suppressor gene product can be detected in the serum of 225 patients with hepatic angiosarcoma with long-term exposure to vinyl chloride, suggesting that p53 gene can be used as one of the monitoring indicators for high risk population of hepatic angiosarcoma. .
5. X-ray examination: some patients with chest X-ray film can show squat elevation or other rare signs, such as right pleural effusion, atelectasis or pleural mass, in the case of angiosarcoma associated with colloidal cerium oxide In the middle, the flat film of the abdomen can be found in the opaque liver, spleen and abdominal lymph nodes.
6. CT scan: Most cases of liver CT scans have abnormal findings, and can find uneven low-density space-occupying lesions and tumor rupture images, and enhance visible calcification.
7. Hepatic angiography: Hepatic angiography can provide valuable information for the diagnosis of the disease, and the abnormal blood vessel morphology, continuous staining around the tumor and central radial light transmission area are highly suggestive of hepatic angiosarcoma.
8. Radionuclide scans showed defects in 70% of patients.
Diagnosis
Diagnosis and diagnosis of hepatic angiosarcoma
Diagnostic criteria
History of exposure to vinyl chloride, often white blood cells, thrombocytopenia, prolonged prothrombin time, abnormal liver function, elevated ALP, hyperbilirubinemia, X-ray, CT, hepatic radionuclide scan found liver occupying and filling defects Equal variation. Burston proposed three diagnostic criteria for reference: 1 pathological morphology like Kupffer cells; 2 angiogenesis tendency; 3 phagocytosis. Diagnosis relies on pathological examination, and liver biopsy is the most reliable.
Differential diagnosis
Clinically, hepatic angiosarcoma is easily confused with diffuse hemangioma of the liver, and it is difficult to distinguish it from hepatoblastoma. In adults, hepatic angiosarcoma must be differentiated from undifferentiated hepatocellular carcinoma. The former is the pathological part, the tumor cytoplasm. It is eosinophilic, the latter is basophilic, and the heterogeneity is obvious. The cancer cells with hepatocyte traits can be identified from many places.
Hepatic hemangioma
Is the most common benign tumor of the liver, can occur at any age, but often in adults with symptoms, more common in women, tumors can be located in any part of the liver, often under the capsule, mostly single (about 10% for multiple), The diameter of the tumor is less than 5cm, but it can be as small as a few millimeters, and some are as large as 30cm. The tumor is less than 5cm in diameter and more asymptomatic. Nearly half of the patients above 5cm have abdominal discomfort, liver, loss of appetite, indigestion and other symptoms. The liver function is generally normal. Ultrasound examination is a typical edge-enhanced echo enhancement zone. Internally, the duct can be seen. Large hepatic hemangioma can be seen with uneven echogenicity. Sometimes calcification is visible. CT contrast agent enhancement or delayed scan is characteristic. The main manifestations were that the periphery of the tumor was over-enhanced and gradually filled to the center with equal density. The MRI scan was on the T1-weighted image of the SE sequence. The tumor showed a well-defined circular-like low-signal region, and the tumor signal was significantly enhanced on the T2-weighted image. And evenly increased, while the normal liver parenchyma signal intensity is significantly attenuated, the tumor/liver signal intensity ratio is significantly increased, and the radionuclide liver blood pool scan is obviously filled, due to the liver Tube tumor was benign, so the patient clinical symptoms and signs are not obvious, the prognosis is good.
2. Primary liver cancer
It is one of the common malignant tumors in China. Most patients with this disease have chronic hepatitis, history of cirrhosis, clinical symptoms such as pain in the liver area, fatigue, anorexia, weight loss, etc. The liver is progressively enlarged, the texture is hard, and the surface and edges are not Regular, often nodular, splenomegaly, ascites yellow or bloody, jaundice can be hepatocellular or obstructive, liver area can be heard and vascular murmur, some patients may have corresponding signs of metastases, such as the collarbone Lymph node enlargement, pleural effusion or hemothorax occurs in pleural metastasis, bone surface appears to protrude outward when bone metastasis occurs, sometimes pathological fracture may occur, paraplegia may occur when spinal cord is compressed, and hemiplegia may occur in intracranial metastasis. Sexual neurological signs, laboratory examination of AFP increase is a relatively specific marker for the diagnosis of hepatocellular carcinoma, which can be differentiated from hepatic malignant hemangioma. Others such as imaging examination can also be differentiated from hepatic malignant hemangioma.
