pediatric hypothalamic hamartoma
Introduction
Introduction to hypothalamic hamartoma in children Hypothalamic hamartoma (hypothalamichamartoma), also known as hypothalamic neuron hamartoma, is mainly found in children and infants and is extremely rare in clinical practice. Sexual precocity, ridiculous epilepsy, some may be associated with other types of epilepsy or behavioral abnormalities. There is no exact statistics on the incidence of hypothalamic hamartoma. The incidence is mostly in early childhood. It has been reported that the average age of onset is 29 months, and women are slightly more than men. In this group, the average age of onset of 17 cases was 20 months from 1994 to March 2000, and the ratio of male to female was 1:1.1. basic knowledge The proportion of illness: 0.003% Susceptible people: children Mode of infection: non-infectious Complications: precocious puberty
Cause
The cause of hypothalamic hamartoma in children
Causes:
There are two types of neuroepithelial tumors, one is formed by the interstitial cells of the nervous system (ie, glial cells), called gliomas; the other is formed by the parenchymal cells of the nervous system (ie, neurons), without a general name. Because of the pathogenic and morphological aspects, it is not possible to completely distinguish these two types of tumors, and gliomas are common. Therefore, neuronal tumors are included in gliomas, and tumors of neuroepithelial origin are the most common. Intracranial tumors account for about 44% of the total number of intracranial tumors.
Pathogenesis:
Hypothalamic hamartoma is characterized by ectopic "brain tissue" at the gray nodule. Pathological sections show that hamartoma is composed of various neurons with well-differentiated and irregularly distributed, astrocytes and ganglion cells. Scattered in the fibrous matrix, wherein the fibrous connective tissue and vascular structure are not obvious. Electron microscopy shows that there are small round bodies of different sizes in the periphery of the neurons. The protrusions contain numerous vesicles and microtubules, and the synaptic structure can be seen. Occasionally, there are myelin axons, which have a large number of high-density secretory granules at the end. Some people have confirmed the secretion of GnRH secretory granules by immunohistochemistry, indicating that the hypothalamic hamartoma has certain neuroendocrine function, neuron-intensive and The numerous synapses suggest that the neurons in this part are extremely active and have a wide range of connections with the peripheral limb system, which may be related to diencephalic epilepsy.
Prevention
Prevention of hypothalamic hamartoma in children
1. Avoid harmful substances (promoting factors)
It is able to help us avoid or minimize exposure to harmful substances.
Some related factors of tumorigenesis are prevented before the onset, and many cancers can be prevented before they are formed. A report in the United States in 1988 compared the international malignant tumors in detail and proposed many external factors of known malignant tumors. In principle, it can be prevented, that is, about 80% of malignant tumors can be prevented by simple lifestyle changes, and traced back. In 1969, Dr. Higginson's research concluded that 90% of malignant tumors are caused by environmental factors," "Environmental factors", "lifestyle" refers to the air we breathe, the water we drink, the food we choose to make, the habits of activities, and social relationships.
2. Improve the body's immunity against tumors
Can help to strengthen and strengthen the body's immune system and cancer.
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.
The most important thing to improve the function of the immune system is: diet, exercise and control troubles. Healthy lifestyle choices can help us stay away from cancer. Maintaining good emotional state and proper physical exercise can keep the body's immune system at its best. Tumors and prevention of other diseases are equally beneficial. Other studies have shown that appropriate activities not only enhance the body's immune system, but also reduce the incidence of colon cancer by increasing the peristalsis of the human intestinal system. Here we mainly understand the diet in preventing tumorigenesis. Some questions.
Human epidemiology and animal studies have shown that vitamin A plays an important role in reducing the risk of cancer. Vitamin A supports normal mucosa and vision. It directly or indirectly participates in most of the body's tissue functions. Vitamin A is present in animal tissues. In the liver, whole eggs and whole milk, the plant is in the form of -carotene and carotenoids, which can be converted into vitamin A in the human body. Excessive intake of vitamin A can cause adverse reactions in the body and -carrot This is not the case with carotenoids, and the low vitamin A content in the blood increases the risk of malignant tumors. Studies have shown that those with low levels of vitamin A intake in the blood increase the likelihood of lung cancer, while those with low blood levels in smokers Levels of vitamin A ingestors have the potential to double lung cancer. Vitamin A and its mixture can help remove free radicals in the body (free radicals can cause damage to genetic material), and secondly stimulate the immune system and help differentiate cells in the body. Ordered tissue (while the tumor is characterized by disorder), some theories suggest that vitamin A can help early carcinogens Invasion mutated cells become reversed the occurrence of the normal growth of cells.
In addition, some studies suggest that supplementation with -carotene alone does not reduce the risk of cancer, but rather increases the incidence of lung cancer. However, when -carotene binds to vitamin C, E and other antitoxin substances, its protective effect. It is shown, because it can increase free radicals in the body when it is consumed by itself. In addition, there are interactions between different vitamins. Both human and mouse studies have shown that the use of -carotene can reduce 40% of vitamins in the body. At E-level, a safer strategy is to eat different foods to maintain a balanced vitamin to protect against cancer, as some protective factors have not been discovered so far.
Vitamin C, E is another anti-tumor substance that prevents the harm of carcinogens such as nitrosamines in food. Vitamin C protects sperm from genetic damage and reduces the risk of leukemia, kidney cancer and brain tumors in their offspring. Vitamin E can reduce the risk of skin cancer. Vitamin E has the same anti-tumor effect as vitamin C. It is a scavenger that protects against toxins and scavenges free radicals. The combination of vitamins A, C and E protects the body against toxins. Better than applying it alone.
At present, research on phytochemistry has attracted widespread attention. Phytochemistry is a chemical found in plants, including vitamins and other substances found in plants. Thousands of plant chemicals have been found, many of which have anticancer properties. The protective mechanism of these chemicals not only reduces the activity of carcinogens but also enhances the body's immunity against carcinogens. Most plants provide antioxidant activity that exceeds the protective effects of vitamins A, C, and E, such as a cup of cabbage. Contains 50mg of vitamin C and 13U of vitamin E, but its antioxidant activity is equivalent to the antioxidant activity of 800mg of vitamin C and 1100u of vitamin E. It can be inferred that the antioxidant effect in fruits and vegetables is far better than what we know. The effect of vitamins is strong, and no doubt natural plant products will help prevent cancer in the future.
Complication
Complications of hypothalamic hamartoma in children Complication precocious
This disease often combined with other congenital malformations, accompanied by single or multiple brain and extracerebral congenital malformations, including cerebellar gyrus, cysts, corpus callosum, multiple fingers, hypothalamic hamartoma can be combined with olfactory bulb hypoplasia, pituitary absent , adrenal gland, thyroid and heart and kidney malformations. The girl showed menarche and breast development, pubic hair growth, and the vulva was full of pigmentation; the boy showed puberty characteristics such as thick penis, hemorrhoids, beard, thick voice, and muscular development.
Symptom
Hypothalamic hamartoma symptoms in children Common symptoms Episodes, smirk, seizures, sorrow, fall, episodes, precocious puberty, mental retardation, generalized tonicity...
Hypothalamic hamartoma has a unique clinical manifestation, most of which occur in early childhood, precocious puberty, ridiculous epilepsy, and some may be associated with other types of epilepsy or behavioral abnormalities.
1. Gelastic seizures: a kind of epileptic seizures, which is characterized by episodes of smirk, which suddenly stops for a few seconds or tens of seconds. There is no loss of consciousness during the attack. It can be dozens of times a day without any The incentives, along with the development of the disease, can gradually appear other types of epilepsy, ridiculous in the beginning of infants and young children, with the increase in age and the frequency of attacks.
2. Precocious puberty: manifested as breast development in infants and young girls, menarche or boy's penis enlargement, pubic hair, hemorrhoids and thickening of the sound, etc. Some authors report that 74% of the hamartomas have precocious puberty, In this group, 53% of children with precocious puberty have increased LH, FSH and estrogen and androgen levels, prematurely into puberty, due to excessive bone development, early growth retardation, but also early development, loss of height development The potential for short stature, precocious puberty may be related to the existence of independent endocrine function units in hamartomas.
3. Mental retardation: This disease is congenital brain development abnormality, so it is often accompanied by poor intelligence.
4. Other types of epilepsy: often accompanied by epileptic seizures and drop attacks.
Examine
Examination of hypothalamic hamartoma in children
Due to the release of the gland hormone from the nervous system in the hamartoma, laboratory tests revealed elevated levels of gonadal hormone.
Neuroimaging is extremely important for the diagnosis of this disease:
1.CT: manifested as saddle back, posterior pituitary stalk, inter-foot pool, mid-brain anterior pool and saddle-upper pool of equal-density space-occupying lesions. Larger volume may have three-chamber anterior deformation due to normal blood-brain barrier. Therefore, there is no enhancement of injection, and there is a clear difference from this part of the true tumor.
2. MRI: It is considered to be the first choice for the diagnosis of this disease. The sagittal and coronal scan of the T1-weighted image can accurately provide the morphology of the tumor and its relationship with the pituitary stalk and surrounding structures. It is characterized by isocortical signals; If the signal is equal or slightly higher, the injection is not enhanced. When the lesion is small or completely located in the hypothalamus, it should be carefully distinguished from the normal papillary body. The large hypothalamic hamartoma can reach 3cm in diameter.
Diagnosis
Diagnosis and diagnosis of hypothalamic hamartoma in children
diagnosis
According to the unique clinical manifestations and neuroimaging features of hypothalamic hamartoma, it is not difficult to diagnose. When children have precocious puberty, idiot-like epilepsy, MRI or CT shows interstitial space-occupying lesions. Consider a hypothalamic hamartoma.
Differential diagnosis
It needs to be differentiated from the following diseases: craniopharyngioma, saddle glioma or germ cell tumor. In addition to abnormal tumor signal or density, the most important thing is that the lesions have different degrees of enhancement and a progressive increase. Hypothalamic hamartoma should be differentiated from hypothalamic astrocytoma, suprasellar germ cell tumor, optic glioma, craniopharyngioma, and saddle septal meningioma. In addition to the difference between tumor signal and density, the most important thing is that the lesions have different degrees of enhancement and progressive increase.
