Pediatric Neuroblastoma

Introduction to Pediatric Neuroblastoma Neuroblastoma (NB) evolved from primitive neural crest cells. Sympathetic chain and adrenal medulla are the most common primary sites. Different ages, tumor sites and different degrees of tissue differentiation make their biological characteristics and Clinical manifestations vary widely, some of which can naturally resolve or transform into benign tumors, but other patients are very difficult to treat and have a poor prognosis. In the past 30 years, the prognosis of infants or early NB has improved significantly, but the prognosis of patients with advanced age is still very poor. There are many factors in NB that can affect prognosis, and age and stage are still the most important factors. basic knowledge The proportion of illness: the incidence rate is about 0.3% - 0.7% Susceptible people: children Mode of infection: non-infectious Complications: anemia, hypertension, dyskinesia

Pediatric neuroblastoma etiology

(1) Causes of the disease

It is an embryonic tumor, mostly located in the cerebral hemisphere.

(two) pathogenesis

NB is derived from the original pluripotent sympathetic nerve cells originating from neural crest. The morphology is blue small round cells. The degree of differentiation, type and transition site of the cells after the transition from neural crest forms different normal tissues of the sympathetic nervous system, including the spinal sympathetic ganglia. Adrenal chromaffin cells, NB histological subtypes are consistent with normal differentiation models of the sympathetic nervous system. Classical pathological classification divides NB into type 3, ie neuroblastoma, ganglion cell tumor, ganglioneuroma, which Three types reflect the differentiation and maturation of NB. The typical NB consists of consistent small cells, about 15% to 50% of cases, there are eosinophilic nerve fiber networks around the mother cells, and another fully differentiated, benign NB. It is a ganglion cell tumor composed of mature ganglion cells, nerve fiber network and Schwann cells. The ganglionoma is between the former two and contains neuroblasts and ganglion cells.

Shimada classification combined with age divided the pathology into 4 subtypes, clinically divided into 2 groups, 4 subtypes including NB (Schwannin less matrix type); GNB mixed type (matrix-rich); GN mature type and (3NB nodular type ( Including less matrix type and matrix rich type), the first 3 types represent the maturation process of NB, while the last type is polyclonal. For NB, cell differentiation is divided into 3 levels, including undifferentiated, poorly differentiated, and differentiated. Type; cell mitotic index (MKI) is also divided into low, medium and high grade 3, Shimada classification comprehensive tumor cell differentiation degree, mitotic index and age, NB is divided into clinical prognosis group (FH) and poor prognosis group ( UFH):

1.FH includes the following types

(1) NB, MKI is low to moderate, age <1.5 years old.

(2) Differentiated NB, MKI is low, aged 1.5 to 5 years old.

(3) GNB hybrid type.

(4) GN.

2. UFH includes

(1) NB, MKI advanced.

(2) NB, MKI is intermediate, aged 1.5 to 5 years old.

(3) Undifferentiated or poorly differentiated NB, aged 1.5 to 5 years old.

(4) All NBs >5 years old.

(5) GNB nodule type.

In pathologically, in addition to HE staining, immunohistochemical examination can be performed to distinguish it from other small round cell tumors. The nerve specific esterase (NSE) is positive at NB, and a typical dense nucleus can be seen under electron microscope. The neurosecretory granules have microfilaments and microtubules arranged in parallel in the nerve fiber network.

Pediatric neuroblastoma prevention

According to the general tumor prevention methods, understanding the risk factors of tumors, and formulating corresponding prevention and treatment strategies can reduce the risk of tumors. There are two basic clues to prevent tumors. Even if tumors have begun to form in the body, they can help the body to improve resistance. These strategies are as follows:

1. Avoid harmful substances (promoting factors) that can 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.

Pediatric neuroblastoma complications Complications, anemia, hypertension, dyskinesia

Anemia, weight loss, high blood pressure, movement disorders, and metastasis can occur.

Symptoms of Pediatric Neuroblastoma Common Symptoms Weight Loss Abdominal Pain Gastrointestinal Symptoms Abdominal Surgery False Chrysanthemum Structure Hypertension Eyeball Highlight Panda Eyelid Cyan Viburnum

The clinical manifestations were related to the primary site, age and stage. 65% of the children had primary tumors in the abdominal cavity. The primary adrenal glands accounted for 40% of the older children, but only 25% of the infants. The other common sites were the chest and neck. About 10% of the cases have unclear primary sites, about 70% of NBs develop before the age of 5, and very few develop after 10 years of age.

1. Lumps in different parts

The most common symptoms are lumps in different parts.

(1) Primary in the abdomen: The sympathetic chain of the adrenal gland and the spine is common in the primary, and the symptoms usually appear when the mass is large. There may be abdominal pain, abdominal circumference, fullness of the back, numbness and mass, stomach. Intestinal symptoms.

(2) primary in the chest: there are symptoms associated with mediastinal compression and respiratory symptoms, such as shortness of breath, cough and so on.

2. Late performance

Patients often have limb pain, anemia, fever, weight loss, eyelid metastasis, orbital metastasis to form a characteristic panda eye, which is characterized by prominent eyeballs, periorbital cyanosis, and other hypertension and mass-related compression symptoms, if any In the spinal canal infiltration, there are movement disorders, incontinence and so on.

3. Transfer route

The main metastasis pathway of NB is lymphatic and blood. About 35% of patients with localized lesions have local lymph node infiltration. The blood transfusion mainly occurs in bone marrow, bone, liver and skin. There may be brain and lung metastasis in the end stage or recurrence, but Rarely, localized lesions in infant cases, localized lymph nodes with localized lymph node metastasis, disseminated lesions were 39%, 18%, and 25%, respectively, but 19%, 13%, and 68% in older children, respectively. %, that is, most of the children of the older age are at the advanced stage of the disease.

Pediatric neuroblastoma examination

Try to obtain pathological biopsy to confirm the diagnosis and classification. To determine the extent of the lesion and clinical stage, bone marrow biopsy or smear should be performed. NB tumor cells should be found in the bone marrow, and 85% to 90% of children with catechol catechol metabolites of the same type of uric acid ( HVA), vanillyl mandelic acid (VMA) increased, LDH in NB can be increased, and is proportional to tumor burden, fluorescence in situ hybridization (FISH) can be used to detect the expansion of tumor cell N-MYC, such as greater than 10 Times, often suggesting a poor prognosis, cytogenetic examination can find 1p- or N-myc amplification.

Selective skeletal X-ray film, X-ray film, bone scan, chest, abdominal CT or MRI, imaging shows that there are often calcifications in the mass, primary in the chest cavity more common in the posterior mediastinal spine, primary in the abdominal cavity More often seen on both sides of the adrenal or posterior peritoneal spine.

Diagnosis and diagnosis of neuroblastoma in children

1. Diagnosis method

Histopathological examination is the most important means of NB diagnosis. Sometimes it needs to be combined with immunohistochemistry, electron microscopy to confirm the diagnosis, imaging examination reveals a tumor that is consistent with the characteristics of NB, and NB tumor cells are found in the bone marrow, and there is a marked increase in catecholamine. Metabolites (HVA or VMA) can also be diagnosed. If pathological diagnosis is difficult, chromosome examination revealed a 1p deletion or N-myc amplification to support NB diagnosis.

2. Diagnostic staging

It should also include diagnostic staging, and the US Children's Oncology Collaborative Component System (CCSG) is as follows:

(1) Stage I: The tumor is confined to the primary organ.

(2) Stage II: The tumor is beyond the primary organ, but does not exceed the midline, and the ipsilateral lymph node may be involved.

(3) Stage III: The tumor exceeds the midline, and bilateral lymph nodes may be involved.

(4) Stage IV: distant transfer.

(5) VIs period: <1 year old, the primary tumor is stage I, II, but there are metastases limited to liver, skin and bone marrow.

Different from other tumors, mainly diagnosed according to pathological examination.

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