Neuroblastoma - a pathological tumor related to tumor changes in the sympathetic nervous system, usually only affects the nerve cells.Neuroblastoma originates in embryos neuroblasts and is considered one of the most frequently developing extracranial and solid neoplasms in children.
Neuroblastoma can be located parallel to the sympathetic trunk in the adrenal region.These tumors observed malignant form and determined immediately after birth with possible congenital disorders.A tumor in the form of neuroblastoma is the second disease frequency of its diagnosis among all the children of tumors that are identified before the age of three.
According to statistics, neuroblastoma ezhegodno affects children in the ratio of 7: 1 million to fifteen years of age.The first clinical symptoms of the disease is not characterized by its specificity, and is regarded as different types of pediatric diseases.
At present, the exact causes of the formation of neuroblasto
Many medical scientists have suggested that neuroblastoma is caused nesozrevaniya embryonic neuroblasts, which are not able to go to the nerve cells, or cells of the adrenal glands.But they continue the process of division and multiplication.Thus, sometimes neuroblasts do not have time to mature before the end of the time of birth and eventually degenerate into malignant neuroblastoma cells.In some cases, neuroblasts continue to proliferate and form tumor and then metastasize to other tissues and organs.
With the growth of the child maturation of these cells is greatly reduced, and the likelihood of developing neuroblastoma is much increased.
Another cause to produce cancerous disease is a mutation in the cellular DNA structure that can be inherited from the parent, and thus increase the risk of development of pathology.Some researchers suggest that familial neuroblastoma arise as a result of inheritance mutagenic, which should depress cancer growth.But most of neuroblastoma are the result of mutations of DNA, and purchased in the early half of life.Such mutations may exist in parental cells that fall into the child's body.Maybe some of the adverse environmental factors are the causes of changes in the structure of DNA, which lead to the development of neuroblastoma.However, they are not exactly confirmed.Also, almost 30% of patients in the cells of the disease is detected and expression of oncogene amplification, and this suggests poor prognosis neuroblastoma.
In addition, there are suggestions that will inherit one type of mutation and the other mutations develop in somatic cells.In the second embodiment, two mutations occur only in the somatic cell.
Initial symptoms of neuroblastoma is not characterized by its own specific symptoms, and simulates various other diseases Pediatrics.This can be explained by the possibility of defeat several tissues and organs, as well as violations of the metabolic nature that cause the development of cancer.
primarily neuroblastoma clinical symptoms depend on the site of tumor growths and metastatic available amount of vasoactive products which are produced by tumor tissue.
Neuroblastoma, which grows and infiltrates, settling in the chest, abdominal, neck and pelvis, can germinate, squeeze the surrounding structures and cause a characteristic set of symptoms.
When neuroblastoma is located in the neck and head, then palpation fails to detect tumor sites, and Horner's syndrome.Penetrating into the chest, there are signs of impaired breathing, squeezing veins and dysphagia.With localization in peritoneal space available palpable tumor foci.With the defeat of the pelvis, revealed violations of acts of urination and defecation.
Neyrooblastoma, mezhvertebralnye sprout in the holes and compressing the spinal cord, causing the characteristic symptoms, such as flaccid paralysis of both limbs, shortness of urination during tense bladder.
Neuroblastoma is also manifested in the major clinical symptoms of a tumor in the abdominal area, swelling, weight loss, anemia and bone pain due to metastases, as well as fever.
mainly patients with neuroblastoma in 35% of cases complained of pain and feverish body temperature jumps to 30%, and weight loss - 20%.
In the propagation anomalies in the posterior mediastinum in patients with marked cough permanent nature, respiratory disorders, dysphagia, and further changes are detected chest.During neuroblastoma bone marrow lesions develop hemorrhagic syndrome and anemia, on entering the retrobulbar space, diagnose the syndrome points and exophthalmos.
for retroperitoneal neuroblastoma tumor ingrowth characteristic of the spinal canal, which resembles a bumpy texture with a stone and impossible to shift it.When pathological tumor extends from the thoracic cavity in the retroperitoneal, it takes the form of a dumbbell.If neuroblastoma metastases gives skin, appear bluish-purple nodes that differ dense content.
clinical picture due to metastases, distinguished by its diversity.For example, the first sign of the spread of neuroblastoma in infants is a rapid increase in the size of the liver, which may be accompanied by skin lesions in the form of a bluish tint, and changes in the bone marrow.In older pediatric patients pain attacks occur in the bones and lymph nodes increases.Also disease can occur with typical signs of leukemia.In these patients developed cutaneous and mucosal bleeding, and anemia.
Violations of metabolic nature which are provoked by tumor growth, lead to the development of symptoms of corporate properties.This is manifested in the form of attacks of sweating, watery diarrhea, hypertension, and pale skin.
Neuroblastoma children develop from immature cells mutants are in the sympathetic nervous system, which is part of the autonomic nervous system.This, in turn, controls the function of somatic organs that independent control available, for example, bladder, intestine, blood circulation and the heart.
Neuroblastoma in children can be formed in any bodies which have the nerve endings of the sympathetic nervous system.Typically, they are located in the adrenal medulla and spine, where there are nerve plexus, ie,in the sympathetic trunk.If neuroblastoma appeared in this section, it can spread to abdominal organs of any of (70%), chest, neck and pelvis.Some tumors are not initially leave the affected area, while others give a lymph node metastases, bone marrow, liver, bones and skin, much less - in the lungs and brain.Neuroblastoma is also characterized by a certain feature that appears spontaneous disappearance of the tumor.
In children and adolescents are diagnosed with neuroblastoma in 8% of all malignant diseases.For example, Germany reported annually nearly 140 cases of the disease among children under fourteen years of age, ie,1: 100000.
Since neuroblastoma tumors are embryonic beginning, and the incidence is marked higher in young children.In general (90%) - are patients before the age of six.About 40% - a newborn and infants.In addition, the disease is most often diagnosed in boys.
Neuroblastoma develops in children as a result of mutations in the embryonic cells that begin to occur before the appearance of the child.In this case, change chromosomes and violated regulation of gene function.But the currently available studies confirm the innocence of neuroblastoma to hereditary factors.Although 1% of children a family inheritance is still present in the presence of the families of cases of the disease.
also can not confidently assert that the impact of the external environment (receiving drugs, alcohol abuse and smoking, a variety of harmful carcinogens) may result in the development of neuroblastoma in children.
Pathological tumor for some time can not be yourself.Many children, when available neuroblastoma, course of the disease are asymptomatic.The tumor can be detected in a child with a planned or random inspection pediatrician, as well as during the X-ray or ultrasound.As a rule, children are claiming their first complaint is when the tumor reaches a considerable size and grow into other tissues and organs, interfering with their work.
All children's symptoms of neuroblastoma may consist of multiple symptoms and depend on its localization and metastasis.First among them is the tumor itself, which fails to probe palpation.Most children have abdominal bloating and swelling in the neck.
Neuroblastoma at localization in the abdominal area, and the adrenal glands, disrupting the child's urinary tract, which leads to abnormalities of the bladder.This symptom is shortness of breath in children appears at the location of the tumor in the departments of the chest and squeezing her lung.But partial paralysis occurs during germination of neuroblastoma in the spinal canal of the spine.
Rare symptoms in children are considered frequent diarrhea and hypertension, which are formed because of the hormones released by tumor cells.When the tumor in the cervical Horner syndrome appears with a characteristic ceasing to eyeball, unilateral pupillary constriction and lowering eyelids.Among the eye symptoms isolated ecchymosis century, as well as a rare manifestation of neuroblastoma in children can be opsoklonusa-myoclonus syndrome.
In the later stages of the disease in children watched a sluggish state, fatigue, general weakness, a feverish rise in temperature without significant reasons, pallor, increased sweating, increased and swollen nodes in the neck and abdomen, constipation, weight loss, as a result of vomiting and diarrhea,abdominal swelling and pain in the bones.All of these clinical manifestations may be due to other diseases, so in order to exclude, you need time to turn to the art and to conduct all necessary inspections.
Neuroblastoma in newborns is growing pretty fast and spreads through the lymph and blood.Basically it penetrates and affects the liver, bone, skin, bone marrow and lymph nodes located remotely from the sites of the primary lesion (the fourth stage of the disease).Furthermore, in infants neuroblastoma may spontaneously disappear, i.e.She programmed cell death.This stage is called the fourth children of S. In such small neuroblastoma patients diagnosed with significant metastases and enlarged liver.Initially, these metastases are soaring, and then compress the lungs and organs in the abdominal area, and then grow to large sizes fatally.Then for no apparent reason, they do slowly disappear, or after the application of low-dose chemotherapy.This regression of neuroblastoma spontaneous nature can be observed in children in the first and third stages of the disease.
When available suspected malignancy, a pediatrician after visual examination of the patient and his family history, assigns various types of laboratory and instrumental methods of examination.It is necessary to exclude or confirm the diagnosis, determine the specific form of neuroblastoma and its distribution in the body.Only after that the doctor will be able to choose the tactics of therapeutic treatment and predict its outcome.
main methods of treatment of patients with neuroblastoma are surgery and chemotherapy.If after such a combined approach to the removal of the tumor remains active cancer cells, then spend radiation exposure of the affected area.Additional therapies may be such as radioactive iodine therapy, high-dose chemotherapy, followed by stem cell transplantation (megaterapiya).
Sometimes some children the process flow neuroblastoma just watching, waiting for the spontaneous disappearance of pathology.In this case, make regular analyzes neuroblastoma tissue microscopy and molecular genetic properties.
The main goal for physicians in the treatment of the child is not only to remove the tumor, but also minimize the risk of complications after the treatment, the consequences after a long time and side deviations.
Thus, all children of the samples after tumor material is separated into three different treatment groups.The first group includes children under supervision in the second, includes children with an average risk, while third are young patients at high risk.
In the retroperitoneal space localized many somatic organs, systems and vessels.These include the kidneys with adrenals, and ureter;pancreas, duodenum, and part of the colon, a part of the abdominal aorta and vena cava, the thoracic duct, stems, lymph nodes and vessels.Therefore, with the defeat of cancer of these organs, retroperitoneal neuroblastoma vysokozlokachestvennoy considered a pathology that is characteristic mainly for small children.This form of the disease occurs in 50% of children two years old and is due to its origin from the cells of the embryo.
In some cases, retroperitoneal neuroblastoma diagnosed another ultrasound of the fetus.Preferably, the tumor begins to develop in the adrenal gland.However, the primary abnormality can be localized in any part, parallel to the spine, and then metastasize to nearby or distant areas.
Retroperitoneal Neuroblastoma is capable of rapid progression, metastasis, and gradual disappearance.The latter phenomenon is observed only at a very early age.Sometimes retroperitoneal neuroblastoma cells spontaneously ripen, and malignancy develops into ganglionevromu.
clinical picture of retroperitoneal neuroblastoma, primarily characterized by significant increase in the abdomen, because of which there are pains and some discomfort in this area.During palpation in patients exhibit a dense consistency tumor, which almost does not move.
at the time Distribution retroperitoneal neuroblastoma appears cough, labored breathing character, broken processes of swallowing and deformed thorax.With the defeat of the spinal canal, patients complain of numbness from the legs, general weakness, partial paralysis form, dysfunction of the bladder and intestines.Then the temperature rises, increases blood pressure, quickens the heartbeat, decreased appetite and weight, there is a concern.When squeezing the blood vessels of the lymphatic and circulatory systems occur in patients with edema and characteristic.
After retroperitoneal neuroblastoma gives lymph node metastases, bone marrow and bone, pain and lameness.And when bone marrow, patients are pale, weak, have been frequent bleeding and decreased immunity.With the penetration of the tumor in the liver, increase it occurs;when skin lesions - reddish and blue spots.
Prediction retroperitoneal neuroblastoma, usually depends on the location of the tumor stage, the patient's age, genetic structure of the cancer cells and primary tumor growth locations.
When the first and second stages of neuroblastoma, most patients have a favorable prognosis.