Pediatric angioreticuloma
Introduction
Introduction to vascular reticuloma in children Angioretinoma originates from the embryonic residual tissue of mesodermal cells and is an intracranial true vascular tumor. It is divided into two types: cystic and solid. The clinical symptoms vary depending on the location and nature of the tumor. Patients with hemangioma or erythrocytosis with other organs, and recurrent tumors in the brain and the brain are called VonHippelLindau disease. basic knowledge The proportion of sickness: 0.004% - 0.01% Susceptible people: children Mode of infection: non-infectious Complications: hydrocephalus ataxia
Cause
Causes of vascular reticuloma in children
(1) Causes of the disease
Vascular reticuloma has a family history of 4% to 20%, and the cause is unclear.
(two) pathogenesis
Vascular reticuloma is due to an obstacle during integration between mesoderm and epithelial tissue components, due to the continuous lack of integration between blood vessels and parenchyma, which is prone to occur in the cerebellum because the embryo participated in the cerebellum at the 3rd month of the embryo. Development, this type of tumor originates from the blood vessels that form the choroid plexus of the fourth ventricle. Sabin believes that vascular reticuloma is caused by interstitial differentiation, producing blood vessels, primitive plasma and red blood cells. In the tumor, the formation of blood vessels is obvious first. The cytoplasmic vacuoles are gradually liquefied, and the result becomes the original plasma, thereby confirming that the tumor cyst fluid is the original plasma.
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 membrane-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 distinction. A large amount of collagen fibers can be seen in meningioma, and the cells are vortex. Arranged, there are no granules in the cytoplasm, no zymogen granules in the adrenal-like cancer cells, no granules with capsules, and only a large number of glycogen granules, 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 huge cysts,
2 cell type: less common, mainly reticuloendothelial cells, few blood vessels, no cyst formation,
3 Sponge type: The main components are blood vessels or sinusoids with different caliber sizes.
4 mixed type: the above types of mixing.
Prevention
Prevention of vascular reticuloma in children
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
Pediatric vascular reticuloma complications Complications hydrocephalus ataxia
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.
Symptom
Symptoms of vascular reticuloma in children Common symptoms Sensory disturbances Reversing neck tonic double vision Increased intracranial pressure Cerebellar signs Ataxia Forced head dysphagia Dizziness
There may be a family history, which can occur in all ages. Children are more common. Tumors can be divided into cystic and solid types. The history of tumors varies, and the growth of solid tumors is slow, which can last for several years or longer. Time, cystic time is short, can be several weeks, months or years, occasionally due to sudden tumor cystic changes or tumor stroke is acute, the posterior fossa tumor is easy to oppress the fourth ventricle, causing cerebrospinal fluid circulation obstruction, The course of disease is also short. When the tumor is located in the cerebellar hemisphere, there is often an increase in intracranial pressure and cerebellar symptoms, accompanied by a forced head position. 90% of patients have symptoms of increased intracranial pressure, manifested as headache, dizziness, vomiting, optic disc edema and Vision loss, vomiting in 80% of cases, vision loss accounted for 30%, neck stiffness accounted for 11%, cerebellar tumor often accompanied by nystagmus, ataxia, walking instability, diplopia, dizziness, vision loss, posterior group of cranial nerves Paralysis, etc., individual cases have medullary symptoms, manifested as dysphagia, throat hoarseness, hiccups, throat reflexes disappeared, diet cough, etc., located in the cerebral hemisphere, depending on the location of the corresponding symptoms and body , May have varying degrees of paralysis, unilateral sensory disturbances, hemianopia, a small number 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.
Examine
Examination of vascular reticuloma in children
1. Blood group examination with erythrocytosis, red blood cell count and hemoglobin amount increased significantly, 9% to 49% of patients with erythrocytosis and hyperhemoglobin.
2. Blood biochemical examination of multiple cysts of the liver can cause damage to liver cells, and transaminase is slightly increased.
3. Cerebrospinal fluid examination of cerebrospinal fluid biochemical phenomenon of protein cell separation.
1. Vertebral angiography is common in the abnormal tumor staining of the posterior inferior cerebellar artery or superior cerebellar artery, which can show tumor nodules.
2. CT is characterized by small tumor nodules in the large cyst, tumor nodules and solid tumors are equal density and uniform enhancement, and the capsule wall is not strengthened.
3. MRI tumor T1 image signal is higher than cerebrospinal fluid is equal T1; T2 image is long T2 signal, and there can be edema around the tumor or small vascular flow, this disease should pay attention to the common posterior cranial sac star Cell tumor identification.
4. Digital subtraction angiography (DSA) MRI shows that children with obvious vascular flow effects 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.
5. Abdominal B-ultrasound can detect multiple cysts in the liver.
Diagnosis
Diagnosis and diagnosis of vascular reticuloma in children
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.
Different from other intracranial tumors, cystic vascular reticuloma should be associated with retinal hemangioma or liver, kidney, pancreatic multiple cysts, and polycythemia if children with astrocytoma (mostly male), if intracranial Increased pressure and cerebellar signs should consider the diagnosis of this disease. Identification, the latter tumor nodules enhanced, scan enhancement uneven, low density, calcification can not be used as the identification point, because both have calcification, solid vascular reticuloma should be differentiated from medulloblastoma, the former Often accompanied by von Hippel-Lindau syndrome, the latter is more common in the cerebellar vermis, for the tumor nodules in the cyst, in the preoperative identification has certain difficulties, the tumor is located in the cerebellopontine angle, need to be differentiated from acoustic neuroma, after Most of them are entities, often with hearing changes, and also need to be differentiated from malignant gliomas and cerebrovascular malformations in the posterior fossa of blood supply.
