hepatic hamartoma
Introduction
Introduction to liver hamartoma Hamartomaofliver is a rare congenital liver tumor-like malformation. In fact, hepatic hamartoma is a feature of tumor dysplasia and is classified as a benign tumor of the liver from a surgical point of view. Its pathological features are hepatocytes as the main component, and contain normal liver tissue with disordered arrangement of bile duct, blood vessel and connective tissue, and abundant connective tissue hyperplasia. basic knowledge The proportion of illness: 0.005% Susceptible people: more common in infants and young children, more common in April to 2 years old Mode of infection: non-infectious Complications: anemia
Cause
Hepatic hamartoma
Causes:
Hepatic hamartoma has been regarded as a tumor that grows along the growth of the hilar structure, rather than a neoplastic tumor. Some pathologists believe that it is likely that the development of primitive mesenchymal cells is abnormal. This abnormal development is likely to occur in In the late embryo, when the liver forms a lobular structure that is connected to the bile duct, Stocker et al. describe that cystic changes in the mesenchymal tissue are accompanied by fluid accumulation due to obstruction, while lymphatic vessels and bile ducts trapped therein cause tumor enlargement, followed by tumor along the tumor. The structure of the portal tube spreads, grows, and forms an island that is trapped in normal tissues. Stocker et al. also pointed out that due to the lack of evidence of meristematic mitotic activity, most of the proliferating growth occurred before or just after birth, but the cyst enlarged into a large mass. In children, but more recent theories assume that these lesions are actually more active than simple growth, in which case an abnormal blood flow supplies perfusion to other normal liver tissue, and the tissue at the site of the lesion is eventually ischemic, reactive cystic Change, observed in observation, some hamartomas have central necrosis and pedicles with normal tissue, which The results support the theory.
Pathogenesis:
In 1956, Edmondson discovered that lymphangioma, hamartoma, cholangiocellular fibroadenomas, cavernous lymphangioma, pseudocyst mesenchymal hamartoma and cystic hamartoma have the same histological features and are uniformly named Hepatic mesenchymal hamartoma, hepatic hamartoma often occurs in the right lobe, close to the edge, the surface is uneven and nodular, typical hepatic mesenchymal hamartoma and microscopic features can be clearly distinguished from other liver tumors Pathologically, it can be divided into two major categories, substantive and cystic. It consists mainly of hepatocytes and interstitial, accompanied by a large amount of fibrous tissue, blood vessels, fat and mucus. The lesions are typically characterized by: huge, border clear or Tumors with envelopes, generally 8 to 10 cm in diameter, although many tumors are polycystic, but single-large cysts are predominant, lesions are mostly stems, and there are reports of torsion, cystic including vascular, lymphatic vessels Sexual and bile duct hamartoma, may have epithelial lining, the gross specimen tissue surface may be brownish gray or hard fish meat, the capsule is filled with serous or mucinous liquid, separated by loose tissue and mucinous tissue, between the cysts The tissue is pale, edematous or reddish brown, resembling normal hepatic mesenchymal tissue. Histopathological features include a mixture of cysts, biliary tracts, hepatocytes, and mesenchymal tissues in different proportions, but also non-cystic, solid hepatic lobe In the case of tumorigenesis, the bile duct is surrounded by connective tissue, elongated and deformed, distributed in the mesenchymal tissue, and the hepatocytes are normal (no immature hepatocytes similar to those in hepatocellular carcinoma), in the mesenchymal tissue of the tumor. Vascular and lymphatic structures can occur.
Microscopically, depending on the origin, the substantial hamartoma is mainly hepatocyte proliferation, bile duct hamartoma is mainly bile duct and fibrillar collagen matrix hyperplasia, interstitial hamartoma is mainly mesenchymal tissue hyperplasia, massive connective The tissues are arranged in a central star shape, the liver cells are arranged irregularly, no lobes are formed, the bile duct epithelium and blood vessels are fibrotic, and Dehnet is observed by electron microscopy. The tumor is composed of loose mucin-like cells, mainly mature interstitial and scattered hepatocytes. The composition of biliary cells, vascular hamartoma is mainly composed of vascular and fibrous connective tissue hyperplasia, cystic can be seen in different sizes of cystic cavity, part of the cyst is lined with a single layer of flat mesothelial cells, part of connective tissue fissure, tumor There are a small number of bile ducts and hepatic cell cords around, and the boundaries between tumors and normal tissues are obvious.
Prevention
Hepatic hamartoma prevention
(1) Improve eating habits. Reasonably arrange daily diet, eat more fresh fruits, vegetables and other foods rich in carbohydrates and crude fiber, increase the proportion of coarse grains and miscellaneous grains in staple foods, and should not be too fine. Change the habit of eating meat and high protein foods. Eat less fatty foods, especially to control the intake of animal fat. Therefore, high-fat, high-protein, low-fiber diets produce many carcinogens, and long-term effects on the large intestine will inevitably lead to an increase in the incidence of colorectal cancer.
(2) Actively prevent and cure intestinal diseases and actively exercise. Prevention of various polyps, chronic enteritis (including ulcerative colitis), schistosomiasis, chronic dysentery, etc., for intestinal polyps should be treated earlier. Colorectal polyps are divided into five categories, namely, adenomatous polyps, metaplastic polyps, inflammatory polyps, hamartoma polyps, etc. Among them, adenomatous polyps are true tumor polyps, which are a kind of colorectal cancer precancerous lesions, so when When an adenoma is found in the large intestine, treatment should be performed, adenoma should be removed and pathological examination should be performed. It is hoped that the benign adenoma stage will be removed to prevent colorectal cancer. If not treated early, the vast majority will become colorectal cancer, and the prognosis is bad. In addition, you should actively treat habitual constipation, pay attention to keep the stool smooth.
(3) Regular inspection. For high-risk populations of colorectal cancer, such as men over 40 years old, familial multiple intestinal polyps, ulcerative colitis, chronic schistosomiasis and family history of colorectal cancer should be regularly checked for vigilance and early warning of colorectal cancer. Symptoms, such as changes in bowel habits, diarrhea, constipation alternate, stool with blood or black stool, stool shape flattened and thin.
Complication
Hepatic hamartoma complications Complications anemia
There are few reports of torsion in this disease, but there is no cirrhosis and no malignant tendency.
1. Pressing the diaphragm upwards can cause difficulty in breathing, which can cause respiratory distress or cardiac insufficiency.
2. Systemic performance can have anemia, weight loss.
Symptom
Hepatic hamartoma symptoms Common symptoms Abdominal mass loss, abdominal distension, constipation, renal capsule hyperinflation, nausea, dyspnea, varicose veins
The clinical features are asymptomatic rapid growth of the mass, the disease is more common in infants and young children, more common in April to 2 years old, usually 5 years before the onset, male: female 2:1, adult incidence is extremely rare, but also reported, There are no symptoms in the early stage, some have abdominal masses at birth, and can grow rapidly with growth and development. When the tumor is gradually enlarged, it can be in the right upper quadrant and the mass, hard, no tenderness, moving up and down with the breathing, late There may be a large painless mass in the abdomen and the resulting compression symptoms. The digestive tract manifests as nausea, vomiting, bloating, constipation and other symptoms of gastrointestinal compression. Pressing the diaphragm upwards may cause difficulty in breathing, and severe respiratory distress may occur. Heart dysfunction, occasional pedicle can occur tumor torsion, necrosis, systemic performance can have anemia, weight loss and so on.
Physical examination showed that the patient's abdomen was extremely bulging, and the abdominal wall varicose veins could touch a huge mass, hard, irregular shape, no tenderness, no movement, no movement, and large liver.
The disease is a rare benign tumor, which occurs mostly in infants and young children. The clinical features are lack of typical specific signs except for the asymptomatic and rapidly increasing abdominal mass. If a huge mass of liver is found, the metastatic cancer is excluded. Protein (-), the patient is generally in good condition, and the benign possibility is large. The disease should be considered. In addition to clinical symptoms and signs, B-ultrasound, CT, etc. are helpful for diagnosis, but usually only suggest liver-occupying lesions, qualitative Diagnosis is often difficult, and the diagnosis is still based on pathological examination.
Examine
Hepatic hamartoma
Liver function can be in the normal range, a small number of AFP is elevated, and the tumor is decreased after removal. The cause is unknown, it is difficult to explain, alpha-fetoprotein is negative, and CA19-9 may be elevated.
X-ray, abdominal ultrasound, CT and MRI are helpful for diagnosis.
1. B-ultrasound examination of the abdominal ultrasound shows a clear echo of the liver without echogenic cysts, which can be isolated or multiple, showing a multi-vesicular (circular or elliptical), thick wall, and no calcification.
2. CT examination showed that the CT showed small vascular masses, capsules and parenchyma with capsules, and the density was lower than that of the liver. It can be seen in the low-density area with large density in the liver, and multiple cystic liquid dark areas can be seen. At present, CT is still a fast and inexpensive method, and at the same time, the anatomical boundary can be well displayed to determine the possibility of surgical resection.
3. Angiography showed no or less blood vessels (small blood vessels between fibrous tissues), lesions invading the surrounding liver parenchyma or invading the inferior vena cava. Hepatic mesenchymal hamartoma is not common, due to less blood supply to the lesion, angiography The help of diagnosis and surgery is small.
4. Radionuclide scanning also helps in the diagnosis of this disease.
5. Abdominal plain film shows that the position of other normal intestinal gas images becomes lower, but calcification may not always be found on the image.
6. MRI is also an effective means. With the development of advanced MRI techniques such as gradient echo imaging and spin echo, MRI has been greatly improved.
Diagnosis
Diagnosis and diagnosis of hepatic hamartoma
Diagnostic criteria
The disease is a rare benign tumor, which occurs mostly in infants and young children. The clinical features are lack of typical specific signs except for the asymptomatic and rapidly increasing abdominal mass. If a huge mass of liver is found, the metastatic cancer is excluded. Protein (-), the patient is generally in good condition, and the benign possibility is large. The disease should be considered. In addition to clinical symptoms and signs, B-ultrasound, CT, etc. are helpful for diagnosis, but usually only suggest liver-occupying lesions, qualitative Diagnosis is often difficult, and the diagnosis is still based on pathological examination.
Differential diagnosis
The hamartoma is a benign tumor, but because the tumor is large and may grow rapidly in a short time, it is easy to be confused with malignant tumors. It should be clinically associated with hepatocellular carcinoma, hepatoblastoma, infant hemangioendothelioma, liver. Identification of adenomas.
1. Hepatoblastoma The hepatic mesenchymal hamartoma needs to be differentiated from hepatoblastoma, but the latter is a solid mass, and AFP is more than 90% positive.
2. Other hepatic cysts Other possible hepatic cysts include biliary cystadenoma, parasitic and non-parasitic cysts also need to be differentiated from hepatic mesenchymal hamartoma, cystic teratoma and simple cysts There is calcification on the abdominal plain film or abdominal ultrasound.
3. Malignant mesenchymal tumors and cysts and malignant histiocytoma are similar to benign hepatic mesenchymal hamartoma in the resected gross specimens, so detailed microscopic examination is required.
