2 English References Hepatic Angiozoon
Overview Hepatic angiosarcoma, also known as hepatic malignant hemangioma, hepatic hemangioendotheliosarcoma, Kupffer cell sarcoma, hemangioendothelioma or malignant hemangioendothelioma, is the most common primary sarcoma of the liver in western developed countries, and it is a primary malignant tumor formed by abnormal proliferation of hepatic sinus cells. It is one of the most common vascular malignant tumors, but it is still rare compared with other liver tumors. Its incidence rate is 10 ~ 20 cases per year in the United States, and 10 ~ 2 cases per year in the United Kingdom. Some cases have been reported in China. Hepatic angiosarcoma mostly occurs in men, and the ratio of male to female is 3 ~ 5.5 1 ∶ 1. The peak age of onset is 60 ~ 70? Years old, a few occur in children (mostly in 3 ~ 5 years old? A one-month-old baby).
Hepatic angiosarcoma is a highly malignant interstitial tumor, and the etiology of most patients is unknown. It was confirmed by fluorescence immunoassay that hepatic angiosarcoma originated from atypical proliferation of hepatic sinus endothelial cells.
The evolution of hepatic angiosarcoma may be mainly in the following five ways: ① the endothelial cells of hepatic lobular sinus changed from atypical proliferation to anaplastic proliferation; ② Hepatocytes proliferate at the initial stage, and then atrophy and disappear; ③ Fibrous tissue hyperplasia in the perisinus space; ④ Progressive sinus dilatation to hematoma formation; ⑤ Sinus wall cells and portal capillary endothelial cells transformed into sarcoma cells.
The main symptom of hepatic angiosarcoma at the beginning is abdominal pain or discomfort. Other common complaints include abdominal distension, rapidly developing liver failure, weakness, loss of appetite and weight loss.
About 25% of hepatic angiosarcomas are complicated with cirrhosis. Rupture of tumor will cause blood abdomen. Hepatic angiosarcoma is easy to be complicated with intra-abdominal hemorrhage and gastrointestinal hemorrhage, and may be complicated with congestive heart failure due to intra-tumor shunt. Patients with hepatic angiosarcoma often suffer from thrombocytopenia and abnormal coagulation due to platelet aggregation in tumor. Massive consumption of platelets in tumor can cause disseminated intravascular coagulation with secondary fibrinolysis, and microangiopathic hemolytic anemia caused by the rupture of red blood cells in tumor blood circulation.
Clinically, hepatic angiosarcoma is easily confused with diffuse capillary hemangioma of the liver and difficult to distinguish from hepatoblastoma. In adults, hepatic angiosarcoma must be differentiated from undifferentiated hepatocellular carcinoma. The former is like the pathological part mentioned above, and the latter is acidophilic and heterogeneous. Many samples show that cancer cells have the characteristics of hepatocytes and can be used for identification.
For patients with localized nodules without cirrhosis, early detection, early surgical resection and postoperative chemotherapy and/or radiotherapy should be sought. The therapeutic effect of hepatic angiosarcoma is not ideal, because patients are often in the advanced stage and cannot be operated. Chemotherapy and/or radiotherapy with 5Fu, vincristine, cyclophosphamide, methotrexate and adriamycin can prolong the survival time. In case of tumor rupture and bleeding, selective hepatic artery embolization or surgical hemostasis can be considered. When thrombocytopenia occurs, platelets can be infused or cryoprecipitated. Liver transplantation is feasible if conditions permit.
Hepatic angiosarcoma grows rapidly, with a high degree of malignancy, a rapid course of disease and few opportunities for tumor resection. 50% patients may have distant metastasis and poor prognosis. Most untreated patients died within 6 ~ 12 months. The cause of death is usually cachexia or liver failure, and may also be due to tumor rupture. Because of the difficulty in early diagnosis, the survival time is generally only 1 ~ 3 years even after operation.
Because the occurrence of hepatic angiosarcoma is related to exposure to harmful chemicals and radioactive substances, it should be avoided. Professionals should do their own protection.
4 cases of hepatic angiosarcoma
5 English name angiosara of liver
6 malignant hemangioendothelioma of liver; Kupffer cell sarcoma of liver; Reticular endothelioma of liver; Hepatic vascular endothelial cell sarcoma; Hepatic angioprotozoa; Kupffer cell sarcoma; Malignant hemangioma of liver; Hepatic angioendotheliosarcoma; Malignant hemangioma of liver; Hepatic angiosarcoma; Coover cell sarcoma
Classification 7-Oncology >: Abdominal tumor > Liver tumor > Liver malignant tumor >: Rare liver malignant tumor.
7. 1 ICD number C22.7
8 classification 2 hepatobiliary surgery > rare malignant tumor of liver
8. 1 ICD number C22.3
Epidemiological hepatic angiosarcoma is one of the most common vascular malignant tumors, but it is still rare compared with other liver tumors. Hepatic angiosarcoma is the most common primary hepatic sarcoma abroad. It has been reported in Japan that 40 cases were hepatic angiosarcoma among 7 autopsies of primary hepatic malignant tumor174/KLOC-0. There are about 25 cases in the United States every year. About 200 cases of hepatic angiosarcoma are reported every year in the world. Domestic reports are mostly individual cases. Wang (1985) and Su Huisi (1987) each reported 1 case, and Chang (2000) reported 2 cases. Yoko (1996) reported 3 cases of primary hepatic angiosarcoma, including 2 males and 2 females 1 case, with an average age of 32.7 years, accounting for 0.69%(3/436) of the detection rate of hepatic malignant tumor in the same period.
Hepatic angiosarcoma mostly occurs in men, and the ratio of male to female is 3 ~ 5.5: 1. The peak age of onset is 60 ~ 70 years old, with an average age of 52 years old. A few cases occur in children (mostly 3 ~ 5 months old babies). Often accompanied by cirrhosis, extrahepatic metastasis (lung, spleen, bone marrow, adrenal gland, lymph, small intestine) is easy to occur.
10 Etiology Hepatic angiosarcoma is a highly malignant interstitial tumor. The etiology of most patients is unknown. Folk et al investigated 168 cases, of which 75% cases were unknown. In recent years, some chemically related hepatic angiosarcomas have been reported abroad, which has attracted people's attention. They are believed to be related to exposure to vinyl chloride. Of the 83 workers in a vinyl chloride factory in the United States, 7 suffered from hepatic angiosarcoma, with an average incubation period of 17. A few can also occur after taking synthetic steroids, estrogen and contraceptives, and the incubation period exceeds 10 years.
Vinyl chloride is a gas produced in the production of polyethylene. Vinyl chloride is converted into mutagenic and carcinogenic metabolites by endoplasmic reticulum enzyme, and then combined with DNA. Experiments show that rats or people exposed to vinyl chloride for a long time can induce hepatic angiosarcoma. Drinking water contains inorganic arsenic, and vineyards use pesticides such as inorganic arsenic and copper sulfate. Hepatic angiosarcoma can occur after long-term exposure. In 1930s and 1950s, 20% thorium dioxide was used as X-ray contrast agent. Thorium contrast agent can remain in liver, spleen lymph nodes and bone marrow for a long time, and its α -ray irradiation 12 ~ 40 years will produce hepatic angiosarcoma or cholangiocarcinoma. There were/kloc-0 cases of angiosarcoma in Hiroshima 36 years after atomic bombing and 3 years after external irradiation with radium ingot for breast cancer. This disease is also seen in patients who have been treated with potassium arsenite for a long time. Other substances that cause hepatic angiosarcoma are radium, inorganic copper and monoamine oxidase inhibitors. Angiosarcoma is also reported in idiopathic hemochromatosis. Related factors of human hepatic angiosarcoma (table 1).
1 1 Pathogenesis The evolution process of hepatic angiosarcoma may have the following five main paths: ① the endothelial cells of hepatic lobular sinus change from atypical proliferation to anaplastic proliferation; ② Hepatocytes proliferate at the initial stage, and then atrophy and disappear; ③ Fibrous tissue hyperplasia in the perisinus space; ④ Progressive sinus dilatation to hematoma formation; ⑤ Sinus wall cells and portal capillary endothelial cells transformed into sarcoma cells.
1 1. 1 Gross examination showed that angiosarcoma often occurred in multiple centers, involving more than two hepatic lobes. Its macroscopic characteristics are many blood-filled sacs, the diameter of which can reach 1 ~ 15cm, and 5cm is common. Tumors are mostly single round lesions, with solid tumors of different sizes, nodular surfaces, clear boundaries and fibrous capsules. The larger lump is spongy, and the largest one weighs 3kg, which may be accompanied by thrombosis and secondary necrosis. The cross section is gray or brown jelly-like shiny tissue, which is interlaced with hemorrhagic necrosis focus, and a large cavity filled with non-condensable blood can be seen in the tumor. Reticular fibrosis and splenomegaly are common in cases exposed to colloidal thorium dioxide or vinyl chloride, but cirrhosis is rare.
1 1.2 Histopathological examination showed that the tumor was composed of spindle-shaped or irregular malignant endothelial cells with unclear boundaries. The cytoplasm is eosinophilic, the nucleus is deeply stained, the shape is long and narrow or irregular, and the nucleoli of the nucleus can be large or small, which is also eosinophilic. Chromosome particles are fine, the nucleus is small, and it is double-stained. Large and abnormal nuclei and multinucleated cells can also be seen, and mitosis is also common. The tumor is scattered in thin-walled veins, and about half of the cases have hematopoietic cells. The earliest microscopic changes of this disease are the enlargement of sinusoidal epithelial cells and the increase of nuclear chromatin. With the development of the lesion, the degree of polychromatic erythrocytosis increases, the hepatic sinus expands, and the proliferation of tumor cells in the hepatic sinus can lead to the atrophy of hepatocytes, the rupture of the hepatic plate, the expansion of vascular cavities, and the formation of cavities of different sizes. At first, malignant cells were supported by a single layer of reticular protein fibers. With the growth of tumor nodules, collagen deposits continuously, and finally completely replaces atrophic liver cells. With the expansion of the vascular lumen, the tumor gradually becomes spongy, and the cells in the vascular lumen can be monolayer, but it is more common to have multiple layers or protrude from the vascular lumen. The cavity wall is rough and uneven, and the inner wall is lined with tumor tissue cells, sometimes in the shape of polyps or * * *, and the cavity is full of blood clots and tumor slices. Tumor invasion of hepatic venules and portal vein branches can lead to the blockage of these vessels, which may explain why tumors are often prone to bleeding, infarction and necrosis. Sometimes tumor cells are densely packed into small nodular solid masses, similar to fibrosarcoma, but fissure-like cavities can be seen in other areas, lined with mild atypical endothelial cells. Hematopoietic phenomena can be seen in hemangiosarcoma caused by thorium.
Immunohistochemical technique can be used to find that tumor cells are positive for factor VIII-related antigens.
In most cases, tumors can invade portal vein or central vein, mostly in tumor nodules, or in distant hepatic lobules, and extramedullary hematopoiesis is common. Iron deposition can be secondary to microvascular hemolytic anemia. In hepatic angiosarcoma induced by colloidal thorium dioxide, it is easy to see that colloidal thorium dioxide is deposited in reticular endothelial cells or dissociated in portal vein, Glisson sheath or hepatic vein wall. Sediments are colorless and refractive, and he stained sediments are usually pink and brownish yellow. Autoradiography can display alpha rays emitted by thorium dioxide, and show short-point trajectories. Thorium can also be measured by energy dispersive X-ray microanalysis. Angiosarcoma caused by thorium dioxide, arsenic or vinyl chloride often leads to cirrhosis and fibrosis in non-tumor tissues. About13 patients have idiopathic cirrhosis, and there is no morphological difference between the tumor caused by pathogenic factors and the idiopathic tumor.
Clinical manifestations of hepatic angiosarcoma 12 The main symptom at the beginning of hepatic angiosarcoma is abdominal pain or discomfort. Other common complaints include abdominal distension, rapidly developing liver failure, weakness, loss of appetite and weight loss. There are several ways of onset:
1. More than half of them are hepatomegaly with unknown causes, accompanied by some digestive tract symptoms, with hepatomegaly, abdominal pain, abdominal discomfort, fatigue, nausea, loss of appetite, weight loss, occasional vomiting and fever as the main symptoms. The course of the disease progresses rapidly, with jaundice and ascites in the late stage, and ascites is pale blood.
2. Symptoms and signs of acute abdomen caused by blood abdomen caused by tumor rupture.
3. A few patients with hepatic angiosarcoma may be accompanied by splenomegaly with or without pancytopenia.
4. Hepatic angiosarcoma often has extrahepatic metastasis, which is spread by blood, and may have symptoms and signs of metastasis to lung, pancreas, spleen, kidney, adrenal gland or bone, with lung metastasis being the most common. Symptoms last 1 week for 6 months, and some last for 2 years.
The liver is swollen with nodules on the surface. Some patients have palpable masses on the liver surface, and most of them have tenderness. Sometimes arterial murmurs can be heard on the surface of the liver. Spleen can be swollen. Ascites is rare, but it may be bloody. Patients with hepatic angiosarcoma are often accompanied by jaundice.
13 complications of hepatic angiosarcoma About 25% of hepatic angiosarcomas are complicated with cirrhosis. Rupture of tumor will cause blood abdomen. Hepatic angiosarcoma is easy to be complicated with intra-abdominal hemorrhage and gastrointestinal hemorrhage, and may be complicated with congestive heart failure due to intra-tumor shunt. Patients with hepatic angiosarcoma often suffer from thrombocytopenia and abnormal coagulation due to platelet aggregation in tumor. Massive consumption of platelets in tumor can cause disseminated intravascular coagulation with secondary fibrinolysis, and microangiopathic hemolytic anemia caused by the rupture of red blood cells in tumor blood circulation.
14 laboratory examination 1. Blood examination: anemia, microangiopathic hemolytic anemia, leukocytosis (65%) or leukopenia (25%), thrombocytopenia (62%).
2. Liver function examination: About 2/3 patients with hepatic angiosarcoma have abnormal liver function: in one group, 65438 000% sodium sulfobromophthalein retention test was positive, 85% ALP was elevated, 60% hyperbilirubinemia, and some patients had elevated ALT. About 50% of patients with hepatic angiosarcoma are accompanied by mild to moderate transaminase elevation.
3. Diffuse intravascular coagulation is a rare complication of hepatic angiosarcoma. Prothrombin time was prolonged in 72% patients.
4.p53 gene: Smith reported that the mutation of p53 tumor suppressor gene product could be detected in the serum of 225 patients with hepatic angiosarcoma caused by long-term exposure to vinyl chloride, so it was suggested that p53 gene could be used as one of the monitoring indexes of high-risk population of hepatic angiosarcoma.
15 supplementary exam 1. X-ray examination: some patients with hepatic angiosarcoma can show diaphragmatic elevation or other rare signs, such as right pleural effusion, atelectasis or pleural mass. In angiosarcoma related to colloidal thorium dioxide, opaque shadows of liver, spleen and abdominal lymph nodes can be found on abdominal plain film.
2.CT scan: Most cases of hepatic angiosarcoma have abnormal CT findings, and uneven low-density occupying lesions and tumor rupture images can be found. Enhanced scanning can clearly see calcification.
3. Hepatic angiography: Hepatic arteriography can provide valuable data for the diagnosis of hepatic angiosarcoma. Abnormal vascular morphology, continuous staining around the tumor and central radial light transmission area all highly suggest hepatic angiosarcoma.
4.70% patients with hepatic angiosarcoma can see the defect by radionuclide scanning.
The clinical symptoms and signs of 16 in the diagnosis of hepatic angiosarcoma are nonspecific, including a history of vinyl chloride exposure, frequent leukopenia and thrombocytopenia, prolonged prothrombin time, abnormal liver function, ALP elevation, hyperbilirubinemia, and liver space occupying and filling defects found by X-ray, CT and radionuclide scanning. Burston put forward three diagnostic criteria for reference: ① pathological morphology is similar to Kupffer cells; ② Tending to form blood vessels; ③ There is phagocytosis. Liver biopsy is the most reliable diagnosis.
17 differential diagnosis Clinically, hepatic angiosarcoma is easily confused with diffuse capillary hemangioma of the liver and difficult to distinguish from hepatoblastoma. In adults, hepatic angiosarcoma must be differentiated from undifferentiated hepatocellular carcinoma. The former is eosinophilic in cytoplasm and the latter is basophilic, with obvious heterogeneity. Many samples show that cancer cells have the characteristics of hepatocytes and can be used for identification.
1. Hepatic hemangioma: It is the most common benign tumor of the liver, which can occur at any age, but it often presents symptoms in adults, especially women. Tumors can be located in any part of the liver, often under the capsule, mostly single (about 10% is multiple), and the diameter of tumors is mostly less than 5cm, but it can also be as small as a few millimeters, and some cases are as large as 30cm, and the diameter of tumors is less than 5cm. The liver function is generally normal, and the ultrasound examination shows a typical echo-enhanced area with clear edges and visible pipes inside. Large hepatic hemangioma can be seen with uneven reticular echo and sometimes calcification. Enhanced or delayed CT scanning is characteristic, which mainly shows that the tumor periphery is over-enhanced and gradually fills to the center with equal density. On the weighted image of SE sequence T 1, MRI showed that the tumor focus was a well-defined circular low signal area. On T2-weighted images, the signal of tumor focus was obviously enhanced and uniformly enhanced, while the signal of normal liver parenchyma was obviously weakened, and the signal intensity ratio of tumor/liver was obviously increased. Radionuclide liver blood pool scanning showed obvious filling phenomenon. Because hepatic hemangioma is a benign lesion, the clinical symptoms and signs of patients are not obvious and the prognosis is good.
2. Primary liver cancer: it is one of the common malignant tumors in China. Most patients with this disease have a history of chronic hepatitis and cirrhosis. Clinical symptoms include liver pain, fatigue, anorexia and emaciation. The liver is gradually enlarged, hard in texture, irregular in surface and edge, and often nodular. Spleen is swollen, ascites is yellow or bloody, jaundice can be hepatocellular or obstructive, and vascular murmurs can be heard in the liver area. Some patients may have corresponding signs of metastasis, such as supraclavicular lymph node enlargement, pleural effusion or hemothorax when pleural metastasis occurs. During bone metastasis, the bone surface can be seen protruding outwards, and sometimes pathological fractures can occur, paraplegia can occur when the spinal cord is compressed, and pathological neurological signs such as hemiplegia can occur in intracranial metastasis. The increase of AFP in laboratory examination is a relatively specific marker for the diagnosis of hepatocellular carcinoma at present, which can be distinguished from malignant hemangioma of liver, and other imaging examinations can also be distinguished from malignant hemangioma of liver.
Treatment of hepatic angiosarcoma 18 For patients with localized nodules without cirrhosis, early detection, early surgical resection, postoperative chemotherapy and/or radiotherapy are needed. The therapeutic effect of hepatic angiosarcoma is not ideal, because patients are often in the advanced stage and cannot be operated. Chemotherapy and/or radiotherapy such as 5Fu, vincristine, cyclophosphamide, methotrexate and adriamycin can be used for treatment, which can prolong the survival time. In case of tumor rupture and bleeding, selective hepatic artery embolization or surgical hemostasis can be considered. When thrombocytopenia occurs, platelets can be infused or cryoprecipitated.
Liver transplantation is feasible if conditions permit.
The prognosis of 19 hepatic angiosarcoma is rapid growth, high degree of malignancy, rapid course of disease development and few chances of tumor resection. 50% patients may have distant metastasis and poor prognosis. Most untreated patients died within 6 ~ 12 months. The cause of death is usually cachexia or liver failure, and may also be due to tumor rupture.
Because of the difficulty in early diagnosis, the survival time is generally only 1 ~ 3 years even after operation.
Prevention of hepatic angiosarcoma Because the occurrence of hepatic angiosarcoma is related to contact with harmful chemicals and radioactive substances, contact should be avoided. Professionals should do their own protection.
2 1 Related drugs: copper sulfate, oxygen, arsenic trioxide, collagen, sodium sulfobromophthalein, thrombin, fluorouracil, vincristine, cyclophosphamide, methotrexate, adriamycin and epirubicin.
Correlation detection of estrogen, monoamine oxidase and prothrombin time
The acupuncture point for treating hepatic angiosarcoma is the "lower diaphragm" of Yangming meridian of stomach tube foot, which belongs to stomach collaterals and spleen; The meridian of Foot Jueyin "holds the stomach and belongs to the liver and gallbladder". According to the principle of "meridian circulation, attending to reaching", this book is selected. ...
The activity of middle tube was the most significant (P0.05). Another person used mice inoculated with S 180 sarcoma as a model to observe the effect of moxibustion on tumor with moxibustion "Zhongwan". ...
The activity of Taicang was the most significant (P0.05). Another person used mice inoculated with S 180 sarcoma as a model to observe the effect of moxibustion on tumor with moxibustion "Zhongwan". ...
The last activity was the most significant (P0.05). Another person used mice inoculated with S 180 sarcoma as a model to observe the effect of moxibustion on tumor with moxibustion "Zhongwan". ...
Ohkura