angioreticuloma
Introduction
Introduction to vascular reticuloma Angioreticuloma is called a hemangioblastoma as a benign tumor, accounting for 1.3% to 2.4% of intracranial tumors. More men than women, about 2:1, more common in young adults. The disease has a genetic predisposition. The clinical symptoms vary depending on the location and nature of the tumor. Patients with hemangioma or erythrocytosis with other organs, and those with recurrent tumors in the retina and brain are called VonHippelLindau disease. basic knowledge The proportion of illness: 0.001% Susceptible people: more common in young adults Mode of infection: non-infectious Complications: hydrocephalus, ataxia, brain edema
Cause
Causes of vascular reticuloma
(1) Causes of the disease
Vascular reticuloma has a family history of 4% to 20%, the cause is unclear. In 2000, WH0 classified it as an unidentified tumor. Because of this disease, it often has retinal hemangioma, pancreatic cyst, pheochromocytoma, etc. These lesions are derived from mesoderm and have a family tendency, so it is considered to have genetic factors. Oertelt et al believe that about 5% of cases have a family history, which is autosomal dominant, male and female have the same penetrance, VHL disease The gene is located on chromosome 3p25-p26, adjacent to 3p13-p14, which contains chromosomal translocations in pure familial renal cell carcinoma.
There are two kinds of vascular reticuloma: cystic and solid type, cyst type accounts for 60%-90%, parenchyma accounts for 10%-40%, derived from perivascular mesenchymal tissue, belongs to cell residual of mesodermal, capsule Containing yellow or yellow-brown liquid, high protein content, most of the cyst wall has a red tumor nodule, protruding into the cyst, under the microscope, the tumor is composed of blood vessels and cells, that is, blood-filled capillary network and blood vessels Reticulated endothelial cells.
(two) pathogenesis
Vascular reticuloma is caused by an obstacle during integration between mesoderm and epithelial tissue components, due to the lack of integration between blood vessels and parenchyma, which is prone to occur in the cerebellar hemisphere (62.3% to 80%) because of embryonic At 3 months, he participated in the development of the cerebellum. It can also occur in the cerebellum or into the fourth ventricle. Occasionally, it is located in the brainstem and cerebral hemisphere. These tumors originate from the blood vessels that form the choroid plexus of the fourth ventricle. Sabin thinks that the vascular network Cell tumors are caused by interstitial differentiation, producing blood vessels, primitive plasma and red blood cells. In the tumor, the formation of blood vessels is first obvious cytoplasmic vacuoles, and then gradually liquefaction, resulting in the original plasma, thus identifying the tumor cyst fluid Primitive plasma, the basic structure of the tumor is a scaffold composed of a cavernous vascular network filled with red blood cells and hemangioblasts, which can cause tissue degeneration, liquefaction and vitreous changes of the blood vessel wall, resulting in cystic changes (70% to 80%). The surrounding glial fibrosis accumulates into the wall of the capsule, and the inner wall is attached with a tumor nodule.
The naked eye of the tumor can be cystic or cystic, and there are also cystic changes of the tumor. The tumor is clear, purple, and the quality is tough. The diameter is different. It is closely related to the brain tissue. The cut surface is dark purple, and the adjacent meninges may have Vasodilatation, brownish yellow hemosiderin can be deposited in the wall of the capsule and tumor nodules, 80% of the tumor is cystic, especially cerebrovascular cystic changes are more common, the capsule contains yellow or tan The liquid is mostly between 10 and 100 ml. The protein content of the cyst fluid is high, and it is easy to solidify into a jelly sample. The protein content of the cyst fluid can be up to 3 to 4 g per 100 ml. It is a tumor exudate, and the inner wall of the tumor capsule is smooth. Vascular-rich tumor nodules, 1/4 tumors are substantial, blood supply is extremely rich, such as sinusoids, red tumor nodules protrude into the sac, about 2cm in size, but some tumor nodules are less than 1cm, and some are even hidden in the sac In the wall, it is not easy to be found. The solid tumors are large. Individual diameters can reach 10cm. They are purple, bright red, yellow, soft, rich in blood supply, easy to bleed, and clearly defined with surrounding brain tissue. Most cases are single. Hair tumor, how many cases can be more Tumors are distributed in different parts of the brain.
Under the microscope, the tumor system is highly rich in young vascular cells and vascular cavities of varying sizes and reticular cells in between, mainly capillaries, with spongy vessels between them, tubes filled with red blood cells, and some are large blood vessels and There are many vascular mother cells between the sinusoids and the vascular network. These cells are of different sizes, polygonal, triangular, round, oval and irregular, with abundant cytoplasm and vacuoles (foam). Contains lipidoids, forming larger foamy cells, also known as pseudo-yellow tumor cells, which are round or elliptical, not deeply stained, meiotic and polymorphic nuclei are rare, occasionally megakaryocytes are formed, and tissues occur within the tumor Denaturation and vitreous changes in the vessel wall, so the tumor often has cystic changes, the capsule size is different, can be fused into a large cyst, and the silver carbonate reticular staining shows many fine silver fibers, which can be seen by many reticular fibers around the cells. The blood vessels and the wall of the capsule are composed of fibrotic glial cells. The abnormalities of the nucleus may occur in the tumor cells, and the nucleus increases and the presence of multinuclear giant cells. If no nuclear separation occurs, it is still not Tumor becomes malignant, the individual tumor tissue were seen scattered in the center of the outer bone marrow, containing positive red blood blasts.
Under the electron microscope, the young capillaries are covered by endothelial cells, and the cell bodies protrude into the lumen. The cytoplasm of the mesenchymal cells contains many reticular substances, and there are many round, thin-film-covered bodies, much like pancreatic cells. The zymogen particles, in addition to the cytoplasm, can also be found in the distribution of the original pulp fibers.
The macroscopic and histological morphology of parenchymal vascular reticuloma is very similar to that of hemangioblastic meningioma and adrenal adenoid carcinoma of the kidney. Electron microscopy can provide a clear basis for differentiation. A large amount of collagen fibers can be seen in meningiomas. Arranged, there are no granules in the cytoplasm, there are no zymogen particles in the adrenal-like cancer cells, and there are no granules with envelopes, only a large number of glycogen particles, which are not found in vascular reticuloma.
Malignant vascular maternal tumors are rare, and the cells are active and densely distributed under the microscope. A large number of cell divisions appear, blood vessels are reduced, and growth rate is fast, forming an adventitial membrane and a vascular endothelial cell malignant tumor.
Vascular reticuloma can be divided into four types according to pathology: 1 capillary type: capillaries, often with large cysts, 2 cell type: less common, mainly reticuloendothelial cells, few blood vessels, no cysts Formation, 3 sponge type: the main components are various blood vessels or sinusoids with different caliber sizes, 4 mixed type: the above several types of mixing.
Prevention
Vascular reticuloma prevention
There is no effective preventive measure for this disease. Early diagnosis and early treatment are the key to the prevention and treatment of this disease.
The etiology of this disease is unknown. Refer to the general preventive measures of tumors, understand the risk factors of tumors, and formulate corresponding prevention and treatment strategies to reduce the risk of tumors. There are two basic clues to prevent tumors. Even if tumors have begun to form in the body, they also Can help the body improve resistance, these strategies are as follows:
1. Avoiding harmful substances can help us avoid or minimize exposure to harmful substances.
2. Improve the body's immunity against tumors can help improve and strengthen the body's immune system and cancer.
(1) Avoid harmful substances: Some related factors of tumor occurrence are prevented before the onset, such as environmental factors, lifestyle, activity habits and social relations.
The focus of our current cancer prevention efforts should first focus on and improve those factors that are closely related to our lives, such as quitting smoking, eating properly, exercising regularly, and losing weight. Anyone who follows these simple and reasonable lifestyles can Reduce the chance of cancer onset.
(2) Improve immunity: The most important thing to improve the function of the immune system is: diet, exercise and healthy lifestyle, maintaining a good emotional state and appropriate physical exercise can make the body's immune system in optimal condition, prevent tumors and It is also beneficial to prevent the occurrence of other diseases.
Complication
Vascular reticuloma complications Complications hydrocephalus ataxia cerebral edema
Can be complicated by hydrocephalus, ataxia, and more with other organs of hemangioma or polycythemia.
If surgery is performed, the following complications may occur:
1. Intracranial hemorrhage or hematoma is not related to intraoperative hemostasis. With the application of surgical microscope and the improvement of surgical techniques, this complication has been less frequent. The wound is carefully hemostasis. Repeated irrigation before closing the skull can reduce or avoid surgery. After intracranial hemorrhage.
2. Cerebral edema and postoperative high intracranial pressure can reduce intracranial pressure with dehydrating drugs, and glucocorticoids can alleviate brain edema.
3. The loss of nerve function is related to the important functional area and important structure of intraoperative injury. The injury should be avoided as much as possible during the operation.
About 90% of patients have symptoms of increased intracranial pressure such as headache, vomiting and papilledema. Common ocular papillary edema causes vision loss for a long time. Most cerebellar tumors have nystagmus and ataxia, and walking is unstable, often with dizziness. Vision, a small number of forced head position, hearing loss, 9 to 11 cranial nerve palsy, hemiparesis and unilateral sensory disturbance, cerebral hemisphere tumors may have different degrees of hemiplegia, unilateral sensory disturbance, hemianopia, etc. depending on the location.
Symptom
Vascular reticuloma symptoms Common symptoms Hiccup sensory dysfunction Ataxia Dizziness Double erythrocytosis Gait instability Neck straight edema Dysphagia
Can have a family history, can occur at all ages, more common in children, tumors can be divided into two types of cystic and solid, the history of tumors vary, the growth of solids is slow, the disease can be several years or more The cystic person is more common in the cerebellar hemisphere, and the course of disease is shorter. It can be several weeks, months or years, and the corresponding symptoms and signs are generated according to the site. The cystic person occasionally has acute onset due to sudden cystic change or tumor stroke. Located in the cerebellar hemisphere, often increased intracranial pressure and cerebellar symptoms, accompanied by forced head position; located in the cerebellar sac in the fourth cerebral ventricle, more early generation of obstructive hydrocephalus and somatic ataxia; at or near the medulla There may be damage to the posterior group of cranial nerves and brainstem; the posterior fossa tumor is easy to oppress the fourth ventricle, causing cerebrospinal fluid circulation obstruction, and its course of disease is also short, 90% of patients have symptoms of increased intracranial pressure, manifested as headache, dizziness Vomiting, optic disc edema and vision loss, vomiting is seen in 80% of cases, vision loss accounts for 30%, neck stiffness accounts for 11%, cerebellar tumor often accompanied by nystagmus, ataxia, gait instability, diplopia, dizziness, Vision loss, posterior group Neurological paralysis, etc., individual cases have medullary symptoms, manifested as dysphagia, throat hoarseness, hiccups, disappearance of throat reflexes, eating cough, etc., located in the cerebral hemisphere, according to the location of the different symptoms and signs, can be There are varying degrees of hemiplegia, unilateral sensory disturbances, hemianopia, etc., and a small number of seizures.
In addition, clinical manifestations are related to pathological type. Capillaries and mixed tumors are prone to form larger cysts. The symptoms of increased intracranial pressure are rapid, the course of disease is short, and it is prone to obsessive head and brain stem symptoms. The composition is higher, the course of disease progresses slowly, the symptoms appear later, the sponge type is prone to intratumoral hemorrhage, the course of disease is short, the symptoms are fluctuating, and it can often suddenly deteriorate. If the bleeding breaks into the subarachnoid space, meningeal irritation may occur. .
Physical examination may have cranial nerve disorders, trigeminal nerve sensation, III, IV, VI brain nerve partial paralysis, 90% of patients with optic disc edema visible in the fundus, a few visible retinal hemangioma or tumor bleeding caused by some manifestations, visceral congenital The disease includes hepatic cysts, polycystic kidney disease, epididymitis, adrenal pheochromocytoma, and epididymal tubular adenoma.
In adults with cerebellar tumor symptoms, in addition to common glioma, the possibility of this disease should be considered, such as the discovery of retinal hemangioma, splanchnic cyst or hemangioma, polycythemia, or family history, the diagnosis can basically be determined However, most patients need CT scan to help diagnose and locate.
CT scans of solid tumors showed round-shaped high-density images, often with uneven density, and cystic lesions showed low density, but generally the density of cysts was high, the edges were not clear, and sometimes high-density plaques appeared to the cystic cavity. Inside, after the contrast agent is injected, the image is enhanced, and there may be a low-density edema around the tumor.
Most of the intracranial vascular reticuloma occurs under the curtain. Adults with symptoms of cerebellar tumor and increased intracranial pressure should consider the possibility of this disease.
Children (mostly male) have retinal hemangioma or liver, kidney, pancreatic multiple cysts, and polycythemia. If there is an increase in intracranial pressure and cerebellar signs, the diagnosis of this disease should be considered, especially the following:
1. Patients with vascular reticuloma have been confirmed in the family.
2. There are true red blood cell hyperplasia and hyperhemoglobinism.
3. Retinal hemangioblastoma is found in the eye.
4. Abdominal viscera found hepatic hemangioma, polycystic kidney, pancreatic cyst and so on.
5. Confirmed diagnosis by cerebral angiography, CT and MRI.
Examine
Examination of vascular reticuloma
Laboratory inspection:
1. Blood examination:
Blood routine examination with erythrocytosis, red blood cell count and hemoglobin amount increased significantly, 9% to 49% of patients with erythrocytosis and hyperhemoglobin, hemoglobin more than 12.5 ~ 17.5g / L, peripheral red blood cells also increased, After the tumor is resected, the red blood cells can return to normal. If the tumor rises again, it often indicates that the tumor has a recurrence, and the reticular hemoglobin is partially increased.
2. Blood biochemical examination:
Multiple cysts of the liver can cause damage to liver cells, and transaminase is slightly elevated.
3. Cerebrospinal fluid examination:
When the intracranial pressure is increased, the pressure can be high, the number of cells in the cerebrospinal fluid is normal, the protein content is increased, and the protein biochemical separation occurs in the cerebrospinal fluid biochemistry. When the tumor is bleeding, the cerebrospinal fluid is mixed with fresh or wrinkled red blood cells.
Film degree exam:
1. Skull X-ray film:
The patients with tumor stroke have blood in the cerebrospinal fluid. The elderly with a history of the skull and the X-ray of the skull can see signs of increased intracranial pressure, that is, the separation of the suture, the thinning of the skull, the increase of the pressure marks, the vertical saddle back, and decalcification.
2. Cerebral angiography:
Vertebral angiography is common in the abnormal tumor staining of the posterior inferior cerebellar artery or superior cerebellar artery. It can show the tumor nodules. It is the most important means of vascular tumors. It can be positioned and qualitative, and can accurately display the blood supply artery and the drainage vein. Can show tumor nodules, cerebral angiography can help to detect tumors less than 5mm, to avoid only the tumor capsules and miss the tumor nodules, vertebral angiography or internal carotid artery angiography can be found in tumor pathological blood vessels, shown as A small group of regular vascular networks, sometimes showing a larger blood supply artery, a substantial tumor can be seen in the petal-like tumor circulation.
3.CT:
Typical manifestations are small tumor nodules in the large sac, tumor nodules and solid tumors are equal density and uniform enhancement, the capsule wall is not strengthened, and the solid tumor CT shows a circular high-density image with uneven density; The cystic person showed low density, higher density than other cysts, and the edge was not clear. The high-density nodules appeared in the cystic cavity, and the density of the posterior capsule wall did not change. The tumor nodules were uniformly enhanced, and low density was observed around the tumor. In the edema zone, according to CT findings, it is divided into cyst nodular type, cyst type, solid type and hydrocephalus.
4.MRI:
The T1 image of the tumor is higher than that of the cerebrospinal fluid. The T2 image is a long T2 signal, and there is a edema around the tumor or a small vascular flow. This disease should be distinguished from the common posterior cranial cystic astrocytoma in children. .
MRI showed that the signal intensity of T1 image of vascular reticuloma was higher than that of cerebrospinal fluid, T2 image changed by long T2, and the tumor nodules were mostly T1 long T2 images. The contour of lesion showed better than CT, and the circumference of tumor was seen on T2 image. T2 edema band.
5. Digital subtraction angiography (DSA):
MRI shows that children with obvious vascular emptying effect should do this examination, which can show vascular mass and blood supply artery, and if necessary, can also be treated with blood supply artery embolization.
6. Abdominal B-ultrasound examination:
Multiple cysts of the liver can be found.
Diagnosis
Diagnosis and diagnosis of vascular reticuloma
Cystic vascular reticuloma should be differentiated from astrocytoma, which has enhanced nodules, uneven scan enhancement, low density, and calcification cannot be used as a distinguishing point. Both have calcification, solid blood vessels. Reticuloma should be differentiated from medulloblastoma. The former is often accompanied by von Hippel-Lindau syndrome, the latter is more common in the cerebellar vermis. For patients with tumor nodules in the capsule, it is difficult to identify before surgery. Bridge cerebellopontine, need to be differentiated from acoustic neuroma, the latter mostly physical, often hearing changes, in addition to the blood supply of the rich posterior fossa malignant glioma and cerebrovascular malformation.
The disease needs to be differentiated from cerebellar glioma, meningioma, cerebellar arachnoid cyst and other diseases.
