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Neuroblastoma

Introduction



Tumor priorities neural (neuroblastoma) and is also called tumor Aljveat nerve, is a cancerous tumor of the type steel, arising from precancerous neurons primary primary (neuroblasts) is the amount of components of the tissues of nervousness, and equal the appearance of cells microscopy usually with the appearance of embryonic cells embryos, and than usual to start growing up nerve tissue in the neck, or thorax, or abdominal cavity, in addition to the pelvic cavity and the cavity of the kidneys, it is more visible and deeply rooted in the abdominal cavity, adrenal gland tissue, and from the most usual cases during the time of onset of symptoms and diagnosis, the tumor to be moving to Menbtha and elsewhere, often to the lymph nodes, liver, or lungs, or bone or bone marrow.

Primitives and a nerve tumor rate ranging from 7 to 10% of all tumors of children, and accounts for more than half of the tumors in infants, and the rate of increase slightly in males than in females, and most cases appear in early childhood, where the diagnosis is about 90% of sick children before the age of six, including the rate of close to 30% are diagnosed during the first year of life, and in most cases, the tumor is present at birth and can not be discovered years ago, and in rare cases have been detected before birth control when growth of embryos using imaging devices, while being diagnosed with less than 3% of the cases at the age of more than ten years, and very rarely appear in adults.

And is the nerve cells, or closeness (Neurons) the main component of the nervous system, which is divided into three sections, the central nervous system (central nervous system), which consists of the brain and spinal cord, and peripheral nervous system (peripheral nervous system), which consists of a neural network that bind the members of the body's central nervous system, and the autonomic nervous system (autonomic nervous system), or independent, which controls the functions of internal organs is voluntary, such as heart rate and digestive processes or blood pressure.
And the autonomic nervous system is divided in turn into two types, the nervous friendly (sympathetic nervous system) or Asambthawayh, and nervous system counterpart friendly (parasympathetic), and composed of bundles of nerve fibers extending along the spinal cord, and clusters of neurons called ganglia concentrated in certain places along their length, and nerve-like cells reside within the marrow (medulla) adrenal gland located above the college directly.

And medical statistics indicate that the tumor priorities nerve arises when the adrenal glands about one third of cases, and the rest of the cases arise when the contract clause of the neural sympathetic nervous chest or abdominal cavity or neck, or the contract clause of the neural neural counterpart friendly area of ​​the basin.

It should be noted that the nerve cells and marrow cells of the adrenal gland will establish the evolution and maturity of primary neurons (neuroblasts) embryos, and it is known that nerve cells cease to divide and thus multiply during the first year after birth (with few exceptions) and that unlike many types of cells that can grow and multiply continuously for tissue regeneration and repair when harmed or other diseases.

And researchers believe that the tumor Aljveat nerve arises when they can not nerve cells the initial embryo from mature to become neurons or serious cells medullary adrenal, and instead continue to grow and divide and multiply in the condition of inadequate, and often do not mature, these cells fully at the birth of the fetus, where the usual concentrations Touselh of neonatal age below three months, and naturally of most of the stage of full maturity and become neurons, adult, or dissolve and disappear, but not tumors, and despite the fact that such residues births may continue to grow tumors composed may be passed to other parts of the body, but nevertheless can mature to the tissues of these tumors neurological or decompose and disappear, but over time, and less possibility of such a development and increase the risk of becoming cancerous tumors.

On the other hand, is a neurological cancer tumor priorities unique in many ways, where pursuing a strange behavior for the behavior of most other tumors of children, as it is one of the few tumors of childhood, which secretes hormones strange body changes occur, such as continuous diarrhea, or changes in the functions of the brain arise Palmtlazmat know about the phenomena associated with the growth of the tumor (paraneoplastic syndromes), such as flicker eyes (opsoclonus) the appearance of rotational movement is involuntary rapid eye, and the phenomenon of muscular convulsions (myoclonus) occurrence of spontaneous seizures or convulsions short-term muscle.

Perhaps the most behaviors surprising that in some cases (melt) cells and simply disappear, that is, they enter the process of annihilation of self (apoptosis), and die, and is a state also known as the death of cells programmed (programmed cell death), and disappears tumor even before start of treatment and sometimes once you start receiving a few of them, and that happens often in infants and young children more than older, and the strange behavior of tumors of childhood, too, that the cells sometimes developed into puberty spontaneously, and stop the division and multiply and evolve to the nerve ganglion cells (ganglion) seriously, leading to its transformation into a benign tumor known as nervous nodal tumor (ganglioneuroma), and not continue to grow and abnormal be eradicated surgically without the need for additional processors.


It is worth noting the presence of tumor last also arise from cells primary neurons is mature and the known tumor priorities nerve ganglion (ganglioneuroblastoma), and of its attributes that it has in some parts of the tissue and tumor benign-like tumor nervous lumpy, and is treated the same methods of treating tumor Aljveat nerve, and therefore the usual surgery when the tumor benign nodal nervous to be examined microscopically to make sure there are no precancerous tumor tissue nerve ganglion-mentioned priorities.











About risk factors for the emergence of tumors of neural primitives



The term risk factors (Risk factors) to the factors and conditions predisposing to increase the likelihood of vulnerability to any disease, such as cancer, when any person, and there are risk factors for each type of tumor, and may include risk factors and genetic any associated characteristics of genes, in addition to factors related to the environment or lifestyle and living conditions, and although the factors on the pattern of living, such as smoking and poor nutrition or alcohol consumption are important factors in many of the tumors in adults, but its impact is none or weak when dealing with tumors of children, and with respect to factors dangerous for the environment, not clear to researchers of any link between the emergence of tumors priorities neurological and environmental factors either before or after the birth, such as exposure to toxic chemicals or contamination of water and air, also did not prove the existence of any link to other conditions, such as the use of X-rays during pregnancy, but that some medical studies on the causes tumors benefit from high-risk for the emergence of tumors priorities nerve when a fetus to the effects of some of the factors and drugs intake both before pregnancy or during, such as the effects of sedative drugs and diuretics (diuretics) and anticonvulsants, and convulsions (anticonvulsants) and hormones, in addition to alcohol abuse and smoking by mothers, but most of these studies are not installed, and accurate statistics to prove convincingly the relationship of these drugs Kmspbh of these tumors, or enhanced medical studies parallel or later can be supported.

It should be noted that there is a recent study conducted in Canada, stating that after the promotion of flour with folic acid (folic acid) and traded in all local markets, significantly decreased the number of children who tumor priorities nerve they have, without adding any conclusions of this study medicine.

On the other hand there are indications of the existence of genetic factors in some children (and if they are rare) increase the likelihood of the emergence of tumor they have, where the observed frequency of infection in children with families already, and a back tumor childhood and some of its members, and according to medical studies that the average ages to the appearance of the tumor in such cases the family of nine months other than the average of the ordinary cases and who is twenty-two months, it can also arise tumors multiple in cases of family and placements are different, such as the two adrenal glands, or more than the subject of the contract nerve Asambthawayh, so Doctors are keen to distinguish between tumors arising in several places at once, and between the tumors in transition, where the first may indicate the presence of genetic factors, while the transmission of the tumor may occur in all circumstances.










About the emergence of tumors of neural primitives



We need to understand how the emergence of cancer cells, knowledge of some initial information and basic genes (genes) and the genetic characteristics, Valmorth is a part of the DNA Alrebonowi (deoxyribonucleic acid DNA) carries the characteristics of specific genetic and has a vital function specific, and an example set of genes eye color and the skin, or blood group, and this acid is a chemical that carries instructions for the system and the life cycle of cells, and the control of all its activities, and is the type of changes anomalies in the DNA of the genes influencing factor in determining the type of disease that may affect humans, and genes are the parts of chromosomes (chromosomes), which can be likened to a band-like linear strand of DNA, containing thousands of genes lined end to end, and the DNA is organized strictly in the 23 pairs of chromosomes, inherits half of each pair for One of the parents.

And there are multiple types and so many of the genes, and each of them vital functions and characteristics of specific genetic, and contains some of the types of instructions and codes of genetic control in the mechanism of growth and division, and thus replication and reproduction for the production of new cells, and from these genes of certain urges and modify operations cell division, called idiomatically Btlieh gene tumor (Proto-oncogenes), and genes other slow and inhibit the division and reproduction, or give instructions to the annihilation of the self-cell, when needed, and called genes inhibiting the swelling (Tumor suppressor genes) .

On the other hand may inherit one some of the changes the anomalous (or defects), DNA from his parents, which explains the appearance of some illnesses are common in some families, and called these changes Balthorat or changes the root of the structure (mutations), which also arise for reasons incomprehensible and unjustified imbalance just like the other images that affect this acid, which can be summarized in the following points:

Distraction of the chromosomes and the exchange sites (Translocation) between the two parts of the DNA, that is, a section of DNA Besbga a homeless HOS Besbga different and asymmetric with him, leading to rearrange programs encoded DNA of the cell with the stability of error and system crashes, context-oriented for the different stages, and this imbalance in particular is responsible for the emergence of some types of tumors of children, such as the Ewing's tumors and some types of leukemia.

Reflection or the coup (inversion), which means that part of the chromosome is inverted, and overlay in reflected light it is still linked to the same chromosome.

Addendum (addition) of any part of the chromosome (chromosome or the whole) has doubled, and found many copies of it to the cell.

Loss or erasure (deletion) of some of the clips Besbga specific DNA.

And from here can Ttsrtun normal cells in the event of any of the mentioned types of imbalance structure of DNA, which in turn paid (directly or indirectly) to either run the forefront of uncontrolled tumor gene (which lose the ability to control cell growth and reproduction Turning so the gene and throw (oncogene) an active subject of the cause Baltsrtun), or lead to frustrate the work of genes inhibiting the swelling, and end up with the emergence of a cancerous tumor, and it is important to know that, and except for genetic disorders that increase the level of risk for the emergence of certain tumors, do not know Scientists and confirmed the real reasons that lead to an imbalance types of DNA, (which trigger or trigger) and that lead to carcinogenesis of normal cells and tumor.

And other words, the cells multiply and divide and grow under the guidance of the program is encrypted Bhamadha nuclear, and receive a signal to stop at a certain point where the available amount desired of cells, depends divide and multiply, and run programs next directed the cells to produce proteins necessary for the cells become very and specialized able to carry out the tasks entrusted to it, ie, that the system of division, growth and employment, stops and starts and is active as a sequential staggered, and free of errors to produce normal cells intact, and there are many ways to block and district progress of this system systematically, for example, If there is a lack of stop sign and did not work for some reason, continue to the cells to divide and multiply unchecked and the mass of abnormal enlarged, or when a failure in the work of genes inhibiting the swelling, which produces a certain protein is responsible for the arrest of the cell when there is damage to DNA , either to fix bugs or to exterminate self when you can not reform, when not working this protein for some reason, continue to cells with damaged DNA in the division, and production of new cells, causing more damage in genes other, which control the growth and development of cells and ends is the emergence of cancer.

In terms of neural tumors Aljveat Although the origins are not known completely, but scientists were able to distinguish many differences between tumor cells and normal primary neurons, which give rise to, which gives some indication of how these tumors, which arise As noted above, while unable to neurons initial maturity to turn into nerve cells, adult or cells of medullary adrenal, and instead continue to grow and divide and multiply in the condition of deficient either embryos or after birth, and it is likely that this deficit arises from the presence of changes abnormal and damages DNA of these cells, and has reported several studies on the presence of loss and erase (deletion) for a section of DNA Babbga No. 1 and this is the loss of more types of changes abnormal cell tumors Aljveat common neural, and believed that the section missing has genes that inhibit swelling or genes control the differentiation of nerve cells the primary, and there, and by less than the loss of a section of chromosome No. 11 and which may contain turn on genes that inhibit swelling, and appears, in some cases, the loss of a section of chromosome No. 14, as reported by research also the presence of added Babbga No. 17 and believed to contain an active gene and throw.

On the other hand is believed by many researchers that the cases of family arising from damages inherited some genes inhibiting the swelling, and need to refer to the changes the anomalous structure of DNA and associated with the emergence of tumors Aljveat nerve when the majority of cases are glitches acquired and casual after birth and during the early stages of the lives of children and not before, hereditary, and is important to remember that there are no risk factors are known and confirmed in most patients these tumors, and no one knows the reasons for the emergence of such imbalances leading to the development of growth of tumor they have, and there is nothing that can be done To avoid the emergence of cancer.



About the symptoms



Symptoms vary phenomenon at diagnosis depending on the subject of the original tumor and the spread and transmission to other sites, whether nearby or distant body, and arises mostly as a result of pressure and effects tumor locus of evolution or to nearby tissues, and show early symptoms are commonly in the form of swelling or mass of overgrown and coherent position the tumor without causing pains to the touch or feel on.

And the usual that the presence of the tumor cavity ventral to the abdominal distension and a child-mother are different, and in some cases, the pressure of the tumor after the increase in growth to the emergence of complications of bladder and bowel, and that the pressure of the tumor mass on certain nerves near the spine can lead to has the ability to move the hands or feet, or pressure on the nerves of certain chest or neck may lead to other symptoms such as ptosis, small pupil of the eyes, while at a pressure of the tumor mass on the superior vena cava, which carries blood from the head and arms to the heart, produces a condition known illness superior vena cava (superior vena cava syndrome), and marks the emergence of the swollen head and chest Baldhirain and the highest, with the change of color of the skin such placements to bluish red, and the emergence of this illness leads to difficulty breathing or swallowing, and may affect the brain and threatens a child's life.

And in some cases may show inflation and swelling placements do not contain cells of a tumor, especially in feet or registered vessels in males, and this is due to the pressure block tumors abdominal or chest on the blood vessels or lymph nodes (and may invade and filled by), which hinders the flow of fluid back to the heart.

And of course there are many of the symptoms arise in the transmission of tumor cells from the subject to arise elsewhere, and the type of symptoms depends on the position of the transition, and often moving primitives nerve tumors to the bone, leading to a sense of pain of varying intensity, and may cause lameness If two men or make the child unable to walk, and sometimes move the tumor to the bone the back and may cause pressure on the spinal cord, causing types of paralysis or numbness, while others may indicate spots skin like bruises and the color purple to a transition of the skin, and may appear dark circles around the eyes as well, and in some cases appear Jahzh eyes, which clearly indicates the movement of the tumor behind her placements.

While the baby is exposed to several risks in the transmission of tumor cells to bone marrow (metastasis occurs to the bone in about 20% of cases), as a result of the shortage in the production of blood cells and low rates to the body, such as the risk of a weakened immune system due to low levels of white blood cells, leading Easy to pick up different infections without the ability to resist, and the risk of anemia due to lack of rate of red blood cells, and then ease the risk of bleeding and lost the ability to repair any cuts or cuttings into the body as a result of low platelets.

It should be noted that the bleeding may occur in rare cases as a result of the emergence of bug Almottagltat excessive consumption (consumption coagulopathy) and is a dangerous illness caused by the rapid loss of blood clotting factors, as a result of excessive fragmentation and dissolution of tissue within the tumor expanding and continuing clot.

On the other hand there are some physical symptoms that appear are not common, such as fever, which arise in about a quarter of cases, and the emergence of severe diarrhea and continuous, high blood pressure and increase the speed of the heartbeat, and redness of the skin and constant sweating, and symptoms are caused by the impact of hormones secreted by tumor cells.

As noted above may arise in some cases eyes flicker syndrome and muscular convulsions (opsoclonus myoclonus ataxia syndrome) and also called the phenomenon of dancing feet and eyes, and show symptoms of convulsions occurrence of spontaneous muscle movements and involuntary rapid eye, twitching and walking without the stability, with the difficulty of speech sometimes, and for unknown reasons, it was noted that priorities nerve tumors that cause this syndrome is less aggressive and dangerous compared to tumors that are not accompanied by this phenomenon.








On diagnosis



When you see the symptoms that can be attributed to tumors Aljveat nerve, and distrust of its existence, the doctors conducted numerous tests and laboratory tests which include blood tests, urine, and take pictures of radiation different from the x-rays X, and X-ray CT (Computed tomography scan ), in addition to imaging MRI magnetic (Magnetic resonance imaging using radio waves, magnetic to put multiple images of the body), and the planning of the bone, and ultrasound, to determine the extent of the rise and throw, and then identify the various aspects of the tumor accurately, and is usually an osteotomy surgery (biopsy), in order to extract a biopsy of the tumor tissue is surgically for examination under a microscope, to determine the kind and dynamic properties.

And is conducting x-rays X-ray chest when you distrust the existence of transmission of the tumor area of ​​the chest and to verify the transmission of the lungs or lymph nodes in the chest, may also be made of the head to verify the move to the bones of the skull, while planning for the bones of detecting the presence of any transfer of the bones of the other body, where it is injection of low-dose radiation from Alticnetyawm 99 (technetium99) accumulate in the places affected bone by the presence of tumor priorities nerve, and the special cameras to show these positions very clearly, is that the planning of the bone also shows no problems with non-cancerous, such as fractures or arthritis, and developments modern action planning by injecting a radioactive substance called dead Ayudubensaal Joinedan and symbolized by the abbreviation M. IBM LG (meta-iodobenzylguanidine MIBG scan) where the stick radioactive particles to this article cell tumor priorities nerve, allowing the creation places the tumor and determine where he moved to the bone or any other part of the body, and this helps in planning treatment and follow-up verification of efficacy and knowledge of the sensitivity of cancer cells, in addition to the possibility of using the same radioactive material and stronger doses to treat the tumor.

And benefit the planning CT normal in the examination of tumor area abdomen and pelvis and chest, while the benefit of planning MRI magnetic, especially in the examination of the brain and spinal cord and in assessing the extension growth of the tumor, as is the use of ultrasonic devices to verify that blocks tumor region of the abdomen and is especially useful to investigate the extent of inflation, the kidneys, as a result of urine retention, which may arise when the swollen lymph nodes, is also used to verify the sensitivity of tumors inside after receiving processors.

And of course target blood tests and urine-finding functions of the liver and kidneys, and measuring the ratios of salts to the body, but explore also the existence of some chemical compounds, which is produced by tumor cells, which secrete nerve cells of natural chemicals known as neurotransmitters (neurotransmitters) control the activities of the Nerves , and defines the main category of vector produced by cells of the nervous friendly vehicles catechol amino (catecholamines), which operates some hormones, and naturally, the body breaks down molecules of these compounds and convert them to outputs of metabolic (metabolites) graduated last command with the urine, and it was noted that in about 90% of the cases, tumor cells Aljveat nerve produces varying amounts of amino compounds catechol that can be investigated with samples of blood, urine, and in fact, some physical symptoms such as diarrhea and high blood pressure and pulse rate return to the effects of excessive secretion of these compounds.

And although the symptoms and blood tests, urine and testing imaging of various types could clearly confirm the presence of tumor priorities nerve, but the only way to be absolutely sure, and then distinguish the type of tumor and its properties precisely, is an osteotomy surgical extraction biopsy of tumor tissue for examination microscopically, using either osteotomy needle, if available, to withdraw a sample of the tumor mass by needle private (and may be used to planning CT during the drag to ensure the accuracy of direction towards the tumor mass), or through surgery Khozaah to extract part of the fabric tumor for examination.
On the other hand, and has traditionally been a biopsy of the bone marrow (Bone marrow biopsy) to investigate the extent of the tumor cells the spinal cord, and so extract a sample of the bone using a special needle is inserted through the skin to the bone, usually hip back where the bone just under the skin and contains large amounts of bone, and then examine the sample under a microscope.

(Please go to a page that tests and laboratory tests for more details on these tests).










On the classification of tumors of neural primitives



When diagnosing tumors priorities nerve, is further analysis and tests to determine the size of the tumor and its properties vital, and ascertain the extent of his move from the position of its inception to the surrounding tissue or to any other sites, and through all the data are classified as grade of the tumor (grading), is determined and classified as progress (Staging), and of course it is necessary to classification of tumors in order to arrange appropriate treatment plans, and to predict therapeutic Palmrdod expected.

And from the usual classification of the degree of tumor Aljveat nerve by the appearance of tissue microscopy to the two main types, tumors Btensj promising or favorable (favorable histology), and tumors Btensj is promising (unfavorable histology), and is rated by comparing the ratios between the numbers of cells emerging and developing countries, and inadequate tissue tumor, and between the numbers of cells that are identical with the neurons of $, which is a tumor with a weave and prepared whenever there is a small number of cells developing and inadequate, and increased rates of neurons adults, and is a tumor with a weave is a promising when there are many of the cells deficient and cells with phases of division and reproduction, and by more than the amount of nerve cells.

With regard to classification of the progress of these tumors, and although there are several systems of classification, however, rolling the use of international classification system (International Neuroblastoma Staging System INSS), which are classified into four grades according to the positions they arise and how their transition, and quantum could be removed surgically, Add to the classification of recurrent tumor:



Degree 1: the occurrence of and subject to eradication (Localized resectable)

Include this category of cases where the main tumor and is limited to the subject of evolution, and can be removed tissues phenomenon is visible in whole surgery, and can be found the remains of microscopic tumor cells at the edges of the subject of surgery, may also contain lymph nodes adjacent to the mass of tumor cells, tumor, and without that there are lymph glands carcinogenesis outside into the tumor.

Degree 2: confined and can not be eradicated (Localized unresectable)

And include this category of cases where the main tumor and is limited to the subject of evolution, but it is not possible to remove the entire tumor mass surgically, either because of the position of the tumor size, or proximity to vital organs. And subdivided into two degrees subgroups:

Grade 2: a tumor where there is one side of the body, (as the spine at a separator between the imaginary side of the body).

° 2 b: the tumor is located next to one of the body, but he moved to the lymph glands in the same side as well as glands adjacent to the tumor, without moving to the other side glands of the body, or to places far away.

Grade 3: local (Regional)

In this category, the tumor has moved from the position of its inception to the lymph nodes nearby, but the other side of the body and confined them, or go to the vital organs nearby, but he did not move to other places far away, or the present area of ​​the center, but it grows about sides, and impossible to remove completely with surgery ..

Degree 4: diffuse and Menbt (Disseminated)

In this case, the tumor has moved from one position to the evolution of the distant lymph glands, or bones, liver or bone marrow or other vital organs, except for positions classified in the class for 4 hours.

4 ° Q, (4 S)

And also called the tumor's degree, and here include cases where the age of the child for at least a year, and the tumor is limited to the original position and upbringing does not go directly to the other side of the body, (and may spread to the lymph nodes in the same side without affecting the glands on the side the other), or the tumor is of limited movement within the liver or skin, or limited to the transfer of bone marrow (a condition that does not exceed the proportion of spinal cord cells Almtsrtunh 10%), and the emergence of direct damage to bone imaging tests.

Recurrent tumors (Recurrent)

This classification means that the tumor had returned and appeared after it was processed, and may return in the same place of origin, where he began, or the surrounding tissue, or returns in another part of the body.









Indicators of disease



In addition to the classification systems of the progress mentioned above, it is important to identify signs and indicators of disease or factors to predict Palmrdod therapy (prognostic factors), through drawn from the data of various tests and analysis, and to assist in the classification of cases and order of treatment plans appropriate, and to predict Palmrdod therapeutic and exploring the extent of tumor response to treatments planned.

And the following indicators are important evidence in the classification of cases and order of treatment plans:

Age of the patient:

It was noted that tumors in children age less than 18 months are more responsive to the processors, and achieve the best therapeutic yield of older children.

Grade of the tumor:

Baltnsj tumors is promising a good indicator of yield better therapeutic, tumor tissue, where a large proportion of nerve cells mature and the less the proportion of defective cells, which on its way to growth, while giving Baltnsj tumors is promising a poor indicator.

The consistency of DNA:

Or ploidy of the DNA (DNA ploidy), where they can measure the amount of DNA each cell using the analysis of census cellular cascade (flow cytometry) or imaging (imaging cytometry), and longer tumor cells double chromosomes (diploid), any consistent, while containing the same amount of normal cell DNA, while the cells are too chromosomes (hyperdiploid) while containing larger amounts without having to double up, and is incomplete chromosomes (hypodiploid) when containing less than the amounts they contain normal cells, called quad-chromosomes (tetraploid) While double quantities of DNA, and according to medical studies that the cells excessive DNA indicate the early stages of the disease, and are more responsive to chemotherapy, and provides cost-effective remedial best, while not giving the cells lacking the DNA and the Quartet, a good indicator of tumor response to treatments standard.

Analysis of cellular genes (Cytogenetics):

Contain normal cells with 46 chromosomes (chromosomes), and are enumerated chromosomes and highlight any imbalance when conducting analysis of genes, cellular, and observed that tumor cells numbered normal chromosomes are more aggressive and resistant to treatments, while those that show the chromosomes of more than the normal number is less aggressive and more responsive to the processors, and that the presence of loss and erase Babbgeyen No. 1 and No. 11, and the presence of added Babbga No. 17 is an indication of the possibilities is promising.

Iceberg tumor gene (Oncogene amplifications):

As noted above, play a leading gene tumor (Proto-oncogenes) play a key role in the operations cell proliferation Bthvezha and urge them and modify the operations division and cell growth, and may pay damages to these genes to the turn of the genes and tumor (oncogenes) could be activated in turn a result of this imbalances and lead to excessive growth and reproduction of cells without controls, in addition to the emergence of many of the properties of tumor growth, and medical research suggests that in some cases of nerve tumors priorities tumor cells contain many copies of the gene and throw abbreviated as n. Mick (N-myc oncogene), and these tumors rapid growth, in particular, and little of cells stages of maturity and adulthood, and noted that the existence of this property is not a good indicator and low rates of recovery standard in patients with tumors carrying this property for the rest of the patients .

Blood serum enzymes:

Explore the analysis of blood serum in cases of tumors priorities nerve on the rates of three compounds, in particular, the most important rates of protein Alhdeden (ferritin) One of the important elements in the metabolism of iron, and rates of yeast with claims of hydrogen salts of lactic acid (lactate dehydrogenase LDH), and rates of enzyme Alanoleezz cells nerve (Neuron specific enolase NSE) and is one of the important enzymes in the metabolism of carbohydrates, where the tumor cells secrete varying amounts of these compounds and produced by the body naturally, and no longer rates rising blood indicative of a therapeutic good yields.








Classification by categories of risk



Based on indicators of disease and age of the child in addition to the interim classification, the classification of tumors of neural primitives into three categories called groups or categories of risk: the risk of a low (Low risk), and moderate-risk (Intermediate Risk), and high-risk (High Risk), and point out that the category of low risk patients may need only to simple treatments and surgery often work alone, while others need to multiple processors and denser.

And such classification is as follows:


Low-risk categories (Low risk)

Low-risk category include the following cases:

Cases of tumors and the administration, confined mainly a viable eradication.

Cases of tumors mainly 2 (a, b), at the age of less than one year.

Cases of tumors mainly 2 (a, b), at the age of more than a year with no iceberg tumor Palmorth n. Mick.

Cases of tumors mainly 2 (a, b), at the age of more than a year with a flood Palmorth tumor n. Mick, but the tumor is of woven and promising.

Special classification of tumors 4 h with no iceberg tumor Palmorth n. Meek, and tumor cells and tumor excessive chromosomes with a woven promising.

Moderate risk categories (Intermediate Risk)

And this category include the following cases:

Cases of tumors mainly 3, at the age of less than one year, with no iceberg tumor Palmorth n. Mick.

Cases, mainly 3, at the age of more than a year, with no iceberg tumor Palmorth n. Mick and the tumor is of woven and promising.

Cases, mainly 4, at the age of less than one year with no iceberg tumor Palmorth n. Mick.

Special classification of tumors 4 h (at the age of less than one year), with no iceberg tumor Palmorth n. Meek, and tumor cells consistent chromosomes or Btensj is promising.

High-risk categories (High Risk)

And this category include the following cases:

Cases, degree 2 (a, b) and at the age of more than a year, with a flood Palmorth tumor n. Mick and the tumor with a weave is promising.

Cases, mainly 3, at the age of less than one year, with a flood Palmorth tumor n. Mick.

Cases, mainly 3, at the age of more than a year, with no iceberg tumor Palmorth n. Meek, and woven into a tumor is promising.

Cases, mainly 3, at the age of more than a year, with a flood Palmorth tumor n. Mick

Cases, mainly 4, at the age of more than a year.

Cases, mainly 4, at the age of less than one year with an iceberg tumor Palmorth n. Mick.

Special cases classification 4 h with a flood Palmorth tumor n. Mick.



On processors



There are treatments for all cases of this type of tumor in children, and most of them are curable to varying degrees, and the dependent child's chances of recovery and treatment options and treatment plans on several factors combined, the most important type of symptoms identified at diagnosis, and the subject of the tumor over the concentration, and vitality and the stage and extent of outside position, and its impact on the surrounding lymph nodes, and the manifestation tissues under the microscope, in addition to the child's age and general health condition, in other words, the treatment plan is arranged according to classification within the categories of risk, and usually follow the standard treatments that have proved their effectiveness.

Line of initial treatment is surgery when the majority of cases, followed by chemotherapy with radiation therapy with or without, and may be conducted operations marrow transplant bone marrow in some cases when you receive doses of therapeutic high, as is the use of certain compounds vital assistance as one of the patterns of treatment is vital, but the treatment plans of course vary depending on the classification of the tumor and classification of cases within the categories of danger and disease indicators mentioned above.



Surgery



A surgery where possible to eradicate the more many as tumor tissue, followed by a start chemotherapy with radiation therapy with or without, and in the case of a tumor in a position to prevent the surgery and can not eradication, is limited to surgical work to draw biopsies of tissue tumor of a different procedure tests, and may be used binoculars to make the abdomen osteotomy if the tumor region of the abdominal cavity.

In most cases, removed the entire tumor mass, especially if wrapped around the blood vessels, a large, leading to full recovery, and may be necessary in some cases re-surgery after receiving doses of two treatments radiation and chemotherapy to investigate the results of treatments and to remove any residue from cancerous tissue if possible, he also received the usual doses of chemotherapy to reduce tumor mass by direct surgical work, and it is important to investigate the surgeon during the operation of any evidence of transmission of the tumor to vital organs, such as the lymph nodes on both sides of the abdominal cavity that are removed and examined for verification, and may also be in many cases, extraction of liver tissue biopsies to check for transmission of the tumor to the liver where it is possible that does not show such a transition and a clear visual tests.

And, of course, may impose the risk of developing postoperative complications, such as the emergence of bleeding and damage to blood vessels, kidneys, or some other member or the nerves, and such complications occur in the proportion of between five to twenty-five percent of the cases, and often arise When the tumor grows inside the room and the blood vessels or nerves.










Chemotherapy



Chemotherapy is the treatment using special drugs known drug chemical anti-cancer, the elimination of cancer cells and destroy them, and so disrupt and undermine the coordinated biological processes within them, and come the main advantage of this treatment in his ability to deal with tumors of mobile and deployed, whereas the radiation therapy or work on the surgical treatment of specific placements administration, confined tumors, its effectiveness and unique due to the fact that cancer cells, in a way, is more sensitive and more susceptible to chemicals than normal cells. And may be used as a treatment only in some cases, or part of a treatment program integrated includes several treatments in common, and the decision is made by using this treatment, the balance between effectiveness and side effects and complications of the future, and between the seriousness of cancer, and of course Vamadaafath and its acceptable compared to the same disease, in addition to the positive therapeutic yield significantly.

And may be called the chemotherapy treatment systemically (systemic); due to movement of drugs, chemicals via the blood to all parts of the body, and its ability to destroy cancer cells wherever notified, and may be used before the direct surgeries when solid tumors to prepare them, and in order to facilitate; Bhzareth and shrink the tumor, what is known as chemotherapy Initial Assistant (Neoadjuvant), may also be used after surgery and eradication of the tumor; to eliminate any cells of a tumor is characteristic may be outstanding, and help avoid the return of cancer, what is known as chemotherapy additive (adjuvant) .

And be dealt with chemotherapy drugs in ways and different channels, some of which are taken orally in the form of tablets or capsules or liquids, and most of the body is injected, the injection of different ways: by intravenous injection, intramuscular injection, injection into an artery or a major injection topically directly under the skin, and if intravenous is the most widely used method, and may use other means to assist in the injection, such as catheters (catheters), which is implanted usually chest and can be used for long periods, and is injected drugs directly into the spinal fluid cerebrospinal surrounding the spinal cord and brain, known as the injection vaginalis, both to eliminate tumor cells, or to protect the central nervous system and brain, and is usually injected through the lumbar spine down the spine, or via a catheter especially planted under the scalp known as portfolio Omaya ( Ommaya reservoir).

And treatment programs consist of several repeated cycles separated by periods of convalescence, and the patient may receive during each session, a combination of several common chemical drugs, or are limited to one drug, by type of tumor and the planned therapeutic approach in each case. And in general is the use of chemotherapy during the time periods prolonged to reduce how much the cancer cells gradually, to the extent that enable the immune system control of any growth and throw, in addition to that space of time between doses are placed in order to achieve the greatest effect on cancer cells, and At the same time give sufficient time to allow cells and tissue normal to recover from the effect of drugs, chemicals, because of the types of drugs, the different effects of varying degrees on the cells and organs of natural sound, especially the cells and tissues rapid growth and heavy reproduction and a permanent replacement, such as bone marrow cells, and blood cells, and cells and tissues of the digestive system, in addition to some vital organs like the liver and kidneys, which in turn leads to complications side effects, which vary in intensity and quality of the drug to another and from one person to another, and of course of treatment to another even for the same person, and depend mainly on the type and dose of the drug user and the interaction of the body about it, and these effects are multiple; include frustration bone marrow (and thus decrease blood cell count), and complications of the mouth and gums (such as inflammation and ulceration, and drought ), and hair loss temporary, constipation, diarrhea, and fatigue, nausea, vomiting, loss of appetite, and feel the skin and skin, and usually take drugs to help and take some preventive measures and support; to avoid such effects and to prevent them and to alleviate it, before and during treatment sessions and after termination.

And in general is the use of chemotherapy in all cases of tumors Aljveat nerve, either by direct action or following surgical termination, and in some cases where the tumor is moved to several places and can not be spread with full surgical removal is the use of drugs, chemical treatment and the initial basic treatment plans.

And habitual use of several combinations of chemotherapy drugs include:
Saakulovosvamaid (cyclophosphamide) or Ivosvamaid (ifosfamide), and cisplatin (cisplatin) or carboplatin (carboplatin), and vincristine
(Vincristine), and Dotxorobaisin (doxorubicin), and Aitoboseid (etoposide), and Tubotikan (topotecan), and Mylvalan (melphalan), and Tinnibuseid
(Teniposide), and generally provide these drugs positive results in whole or in part, the ratio of more than two thirds of cases, and the most combinations commonly used combination drugs Saakulovosvamaid and Dotxorobaisin and vincristine, alternating with a real estate cisplatin and Aitoboseid, and may add other drugs to these combination to achieve long-term response in cases of advanced tumors, in addition to the presence of some of the other combinations.

(Please go to chemotherapy, to view more details about this treatment and methods of its management and its complications).










Radiation therapy



Radiation therapy is the treatment using the different applications of ionizing radiation (ionizing radiation), to destroy cancer cells and shrink tumors, both using the elements and isotopes, or by using a stream of radiation, revived and high-energy, x-rays, or radiation such as gamma rays, or stream of neutrons or protons, and the effectiveness of the radiation is concentrated in its ability to undermine and break up the DNA of tumor cells, and is a vital and fundamental article of the various cellular functions, leading to elimination.

Radiation therapy is topical treatment, and is divided into two types; internal (Internal), radioactive elements are grown directly into the tumor tissue, or close to it, either temporarily or permanently, and external (external), where the broadcast radiation from the radiation machine shed the positions of the tumors, and may be used both types to address some types of tumors, while the use of external radiation only to the tumors priorities nerve, and of course, and to avoid the effects of radiation, is to take preventive measures during radiation therapy, to protect tissues and organs natural sound field treatment.

And may be the use of radiation therapy alone, treatment only, or jointly with treatments other tumors, and may use a substitute for surgery as initial therapy, when some solid tumors, may also be used by the direct action surgical known as the treatment initial Assistant (neoadjuvant therapy), order to reduce the size of the tumor; to facilitate the eradication, or is used after excision surgery as a treatment additive (adjuvant therapy); in order to eliminate any tumor cells is characteristic may be outstanding.

And radiation treatment complications and side effects associated with, linked to the overall locus of treatment, and result in general from affected cells and tissues rapid growth and replacement, and from these effects: complications of skin and skin, and fatigue, and infections and dry mouth, and hair loss , nausea, intestinal problems, and thwart the bone marrow, and is taking some preventive measures and support, and taking medication assistance; to avoid such effects and to alleviate it, and on the other hand and to avoid complications later of radiation therapy in children is keen plans therapeutic use to avoid whenever possible.

And according to medical studies that the combined use of Alajan chemotherapy and radiation in some cases provides better results than only chemotherapy alone, and the habitual use of radiation therapy in infants classification on 4 h when the emergence of difficulties breathing caused by swelling of the liver, is also used to ease the pain When cases of advanced tumors.

As the tumors are developed using dead material Ayudubensaal Joinedan MI BG (meta-iodobenzylguanidine MIBG) above and used at diagnosis, but they are molecules with a high radiation dose, which can eliminate the tumor cells, which stick to it.

(Please go to radiation therapy, to view more details about this treatment and methods of administration).










Marrow transplant bone marrow or stem cell transplant



Require treatment plans in some cases, particularly the category of high risk use of high doses and extensive drug chemical, which in turn affects the bone marrow cells and destroys them, necessitating the need to resort to marrow transplant bone marrow, in parallel with the treatments received, and following a glance for these operations:

Valenkhaa marrow is the spongy tissue soft, known as the spinal cord of red and located within the part of the bone called bone spongy, and is the main function in the production of blood cells, and is composed of cells mutant (turn into blood cells), and fat cells, and tissues help the growth of blood cells, bone marrow and there in almost all the bones in infants, while Prior to puberty, is concentrated mostly in the flat bones, such as skull bone, and shoulders, and ribs, and pelvic bones, and joints of the arms and legs.

And cells at the beginning of the configuration (initial) called cells Aboriginal or stem cells (Stem cells), and is the division's self-multiply, producing stem cells, new, or you through a series of divisions, cumulative, and the phases of growth of successive production of cells of different blood (blood cells white and red and platelets), which continues to stages of growth and maturity within the bone marrow.

And comes the need to perform marrow transplant bone marrow (Bone marrow transplant), or transplantation of stem cells (Stem cells transplant), while unable to bone on the performance of its functions, and result in the deficit either by the effects of the cancer itself, which makes him either a product of cells of a tumor abnormal , or a product of small numbers of blood cells, or resulting from the effects of drugs, chemical and radiation therapy severe, it may be necessary to eliminate cancer cells, (especially when the tumors of blood and lymphatic tumors, and some types of solid tumors), follow the treatment programs is strong, dose extensive lead to frustration and the destruction of cells of the bone itself, and hence the target of transplantation, replacement of stem cells spinal cancer patient, or frustrating treatments, cells intact and healthy, capable of growth and reproduction and the production of blood cells, and include possible sources to derive stem cells and used for transplantation, bone marrow and blood circulation, either from the patient himself or from a donor, and recently conducted studies to be drawn from the umbilical cords of newborns.

And planting operations are divided into two types according to a source of stem cells, self-transplantation (Autologous) where these cells are derived from blood or the patient's own marrow, and gay culture or lineage (Allogeneic) where a pool of blood or marrow donor, and donor selection is appropriate, after analysis of tissue specific blood, known analysis antigens leukocytes (human leukocyte antigens HLA), and aims of this analysis, matching blades genetic protein antigens, produced by white blood cells, and are special proteins Highlight the cells self from foreign bodies such as bacteria or viruses , and the success of agriculture depends on the extent of tissue compatibility between donor and recipient, and is a perfect fit, which brings together six groups of these proteins, and the donor twin brother is the ideal ADSL, followed by the brothers, then a parent, a donor or a non-kin of the patient.

The spinal primary source of stem cells, and when using the implant self, is to draw the amount of marrow of the child, and is processed to eliminate any cancer cells, and then kept under the freeze, and after completion of receiving the treatment intensive, and that would destroy the bone marrow, are spinal implant processor.

When using the implant gay, is to draw the amount of marrow donor, after the tests and analyzes different; to make sure that his general health and free from infectious diseases, and is the selection and collection of stem cells separately, and after the stages of creating a patient for transplantation, giving doses high chemical drugs with or without radiation therapy, in order to eliminate cancer cells, and thwart the immune system to the lowest degree, is implanted stem cells selected.

And is extracted stem cells from blood circulation, and know in this case stem cells peripheral or periodic (peripheral stem cells), and the quantity is usually sufficient to collect and use for transplantation of self becomes a practice, when there is the possibility to urge the bone marrow in the patient, the excretion within the blood circulation in sufficient quantities for transplantation, but when gay culture, will be gathered from the circulation of the donor; the blood passes through the filter of the machine, the separation of these cells separately.

On the other hand transplants involve the complexities and complications of different course, such as the difficulty of finding the matching donor, and complications associated with the process, the effects of chemotherapy and radiation treatments, and the risk of transplant rejection, and failure of cells cultured in the stability and reproduction.

(Please go to Page marrow transplant bone marrow to view more details about the transplantation).




Bioremediation



Is the use of certain types of vehicles from vitamin derivatives (a) (retinoids) as drugs to support priorities in the treatment of neural tumors, and to help identify elements of cell differentiation (differentiating agents), and are believed to help some of the cells to differentiate and reach adulthood naturally as cells, and have found that these vehicles drive cell tumor grown experimentally to continue growth stages to mature into the cells like for show nerve cells, and according to medical studies that prolonged use of one of these compounds, known acid, vitamin (a) 13 (13-cis-retinoic acid ), reduces the risk of relapse and the return of cancer in cases of metastatic tumor classification and Almenbt 4, after the use of intensive treatment of high doses of chemical and stem cell transplants, and recommends the most treatment plans to receive doses of the drug for six months after the end of the processor core, and of course there are side effects associated with taking this drug, and the emergence of the most common dry mucous membranes, especially the lips.










On the processors according to the classification tumor



Categories of low risk: generally not be given additional management after surgery in children category of low risk, and often infants are sidelines is cautious when there are remnants of a tumor after surgery, where often disappear residues spontaneously, except in cases of those who appear to have symptoms associated with, such as pressure tumor mass on the spinal cord or the presence of respiratory problems, where they can receive doses of chemotherapy to treat these symptoms and management, are also receiving chemotherapy if the tumor tissue can be removed surgically less than half the tumor mass, while the infant classification on 4 h of those does not appear to have any symptoms may be the sidelines cautious without treatment as often disappears cancer spontaneously, and the most combinations of chemotherapy used in this category combination drugs Saakulovosvamaid (cyclophosphamide) carboplatin (carboplatin), and Dotxorobaisin (doxorubicin) and Aitoboseid (etoposide), It is possible to use other combinations of course.

Moderate risk categories: not often receive chemotherapy in cases of moderate risk in four to eight sessions (ie, between 12 to 24 weeks) postoperatively either before or after it ends, and a combination of drugs used by groups such as low risk, and may be necessary surgery the next or to receive radiation therapy, where in most cases, and after the end of the surgery and remove the tumor and following the receipt of other therapies, is a second surgery to investigate the efficacy of treatments used, and in order to remove more tissue tumor, if any, even if this is available at some cases, and may follow the use of radiation therapy again.

High-risk groups: The use of intensive treatments with high-risk situations include receiving high doses of chemotherapy, including the operations of marrow transplant bone marrow, in addition to the surgery and radiation therapy, and are in most cases, the use of bioremediation to receive doses of acid, vitamin (a) 13 (13-cis retinoic acid) for a period of six months after the treatments.

And treatment plans can be summarized as the interim classification as follows:

Almnhsr tumor eradication and disposable:

May follow one of the following treatments of the cases in this category:

Surgery to remove the tumor.

Surgery with chemotherapy added immediately after.

Surgery with radiation therapy.

Almnhsr tumor and non-viable root:

Plan consists of initial treatment is usually surgery, to eradicate the more how much tumor tissue as possible, directly followed by chemotherapy, and surgery may be the next to remove any remnants of the tumor tissue, and then direct radiation treatment.

Residual tumor (Regional):

And here the treatment plan depends on the age of the child:

If the child is less than a year, treatment may include surgery to remove the tumor and then chemotherapy.

And that the child is more than a year, the track following the treatment plan:

Surgery to remove the tumor.

Surgery followed by chemotherapy.

Chemotherapy with or without radiation therapy, to reduce and limit the tumor, then surgery is next.

A combination of several chemical drugs.

Radiation therapy.

Metastatic tumor and Almenbt (Disseminated):

And in this category may follow one of the following treatments:

A combination of several chemical drugs with surgery or without, and with or without radiation therapy.

High-dose chemotherapy followed by transplantation of stem cells.

Classification 4 h (4s):

Than usual to be the treatment of children in this category by category of risk.

Recurrent tumors (Recurrent):

Tumor recurrence may occur in short periods, or during the years of initial treatment, and when you return is a comprehensive assessment of the situation, to determine the focus of the tumor, and the area of ​​his presence, and degree and severity or excesses, and adopt the treatment plan depends on several factors, the most important Previous classification by category of risk, and the subject of the tumor and its extension, in addition to treatments that have been followed in the past and the special considerations of each case individually, and as the case may be the use of chemotherapy or surgery or radiation therapy, and sometimes may recommend treating team as soon as the prudential supervision of the situation, and if the tumor cells interact with particles of radioactive material to the dead Ayudubensaal Joinedan MI BG
(Meta-iodobenzylguanidine MIBG) above and used at diagnosis, may be used high doses of radiation to eradicate the tumor but it often requires a transplant of stem cells after as the radiation doses are very high.
May also be used to receive vital treatment doses of vitamin acid (a) 13 (13-cis retinoic acid) upon the return of these tumors, and on the other hand is still an ongoing clinical studies for the development of new therapeutic approaches for this category.









Returns on the therapeutic



There are good opportunities for the healing of many sick children in this tumor after receiving processor intensive and appropriate, and improve opportunities significantly in the cases of tumors administration, confined, and in children age below the year, even with tumors of crossover and advanced, and with some of the properties of the promising, and may end tumors and disappear with little treatment and sometimes without treatment, while the fall opportunities ratio stunning in children older tumors crossover and advanced, and more likely to return tumors after successful treatment of primary and, unfortunately, up to about 70% of the cases that are more than for year are diagnosed and the tumor has moved from a position of growing up, especially to the lymph nodes or liver or bone or bone marrow.

In terms of returns, therapeutic, according to medical statistics that the cure rates for the five-year standard than normal children more than 85% when the age group under one year, while down to about 55% when the category between years to 4 years, and down to about forty percent when the category that are more than 5 years, (five years indicates the rate of recovery standard to the proportion of patients who live at least five years since diagnosed with cancer specific, and of course many people recover fully and live much more, and this rate is usually used as an indication of when the statistical cases of cancer).

On the other hand, and compared with categories of danger, according to statistics that the cure rates for the five-year standard of about 95% of the category of low risk, and range from the category of danger moderate between 85% to 90%, while the fall in the category of high risk to about 30% , and show the same ratios for the years almost standard and its periods of more than ten years, with an approximately 95% in low-risk category, and about 80% in the moderate-risk category, and reduced the cases of extreme danger to around 25%.










After the treatments



Necessary to conduct tests periodically comprehensive last for several years after the end of treatments, in order to investigate any signs of tumor recurrence, as well as to monitor complications and side effects of different treatments received, whether immediate or delayed, which may appear years later (please go to a page of late complications of treatments for tumors For more details), and include these tests in addition to clinical examination flour, laboratory tests and imaging studies and X-rays of different, especially for the position of the emergence of the original tumor, and are made scheduling a specific time according to the special considerations of each case separately, and it is very important of course notify the medical team in case of any symptoms or complications may appear, is to take the necessary medical procedures and to achieve effective treatment without delay, whether the complications of treatment or relapse.

On the other hand, it should be noted that in rare cases, and for reasons still unknown, arising immune reaction towards nerve tissue natural, and starts the immune system attacking nerve cells healthy, and is suffering from some patients of the delayed development muscular and kinetic and difficulties to speak and mental disabilities and behavioral problems, and may be required to receive additional treatments steroidal compounds or other hormones to thwart the immune system in some cases when the tumor zones, neck and chest, who may suffer from the appearance of eye problems or muscle convulsions.
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