Neuroblastoma is a cancer that can arise mostly in the adrenal gland or, less commonly, in the sympathetic chain, incl. Retroperitoneum, chest or neck. The diagnosis is confirmed by biopsy. The treatment may consist of a surgical resection, chemotherapy, radiation, high-dose chemotherapy with stem cell transplant, cis-retinoic acid and immunotherapy exist.

Neuroblastoma is the most common childhood cancer. Approximately 90% of cases occur in <5 years in children. Most neuroblastomas occur spontaneously, but 1 to 2% appear to be inherited. Some markers (z. B. MYCN oncogene amplification, hyperdiploidy, histopathology) appear to correlate with the progression and prognosis. Although MYCN amplification is associated with advanced disease and unfavorable biology, it is also predictive of survival, even without high-risk features. MYCN amplification occurs in approximately 20% of cases.

Neuroblastoma is a cancer that can arise mostly in the adrenal gland or, less commonly, in the sympathetic chain, incl. Retroperitoneum, chest or neck. The diagnosis is confirmed by biopsy. The treatment may consist of a surgical resection, chemotherapy, radiation, high-dose chemotherapy with stem cell transplant, cis-retinoic acid and immunotherapy exist. Neuroblastoma is the most common childhood cancer. Approximately 90% of cases occur in <5 years in children. Most neuroblastomas occur spontaneously, but 1 to 2% appear to be inherited. Some markers (z. B. MYCN oncogene amplification, hyperdiploidy, histopathology) appear to correlate with the progression and prognosis. Although MYCN amplification is associated with advanced disease and unfavorable biology, it is also predictive of survival, even without high-risk features. MYCN amplification occurs in approximately 20% of cases. Tips and risks Neuroblastoma is the most common childhood cancer. Neuroblastomas can in the belly (about 65%), chest (15-20%), neck, caused the pelvis or elsewhere. Neuroblastomas are very rare as primary CNS tumors. Most neuroblastomas produce catecholamines, which can be detected in urine as increased degradation products. Neuroblastoma cause severe hypertension in general, as these tumors secrete usually no epinephrine. The Ganglionneurone are fully differentiated, benign variant of neuroblastoma. Approximately 40-50% of children have a localized or regional disease at diagnosis, 50-60% have at this point already metastases. Neuroblastoma can metastasize bone marrow, bone, liver, lymph nodes, or, more rarely, skin or brain. Bone marrow metastases can cause anemia and / or thrombocytopenia. Anemia also occurs occasionally when a hemorrhage in these highly vascular tumors caused a rapid drop in Hb. Symptoms and signs The symptoms and signs of neuroblastoma depend on the seat and stage of the primary tumor. The most common symptoms are abdominal pain, discomfort and bloating because of abdominal tumor mass. Certain symptoms can result from metastases. These include bone pain due to widespread bone metastases, periorbital ecchymosis and exophthalmos due retrobulbar metastasis, as well as abdominal distention and respiratory diseases due to liver metastases especially in infants. Children with anemia may have pallor, and those with thrombocytopenia petechiae may have. Children sometimes present with focal neurological deficits or paralysis if the tumor collapses directly into the spinal canal. You can also with a paraneoplastic syndrome stand out like a cerebellar ataxia, opsoclonus-myoclonus, watery diarrhea or arterial hypertension. ROHHADNET (rapid-onset obesity with hypothalamic dysfunction, hypoventilation, autonomous dysregulation and neuroendocrine tumors) is a very rare disease that can be associated with ganglioneuroblastomas and ganglioneuromas in abdomen and lungs. Diagnostic CT / MRI biopsy Sometimes bone marrow aspirate or biopsy and urine tests on Katecholaminabbauprodukte In the prenatal routine ultrasound examination is sometimes discovered a neuroblastoma. Patients who present with abdominal symptoms or a tumor mass should receive an ultrasound or an abdominal computed tomography or MRI. The diagnosis of a neuroblastoma is then confirmed by biopsy. Alternatively, the diagnosis can be made even without a biopsy or surgery of the primary tumor, will be provided by the presence of characteristic of cancer cells in the bone marrow or Sternumbiopsie or due to increased Katecholaminabbauprodukten in urine. These diagnostic methods are used infrequently but can be useful in situations where biopsy and / or surgery because of patient or tumor characteristics is classified as high risk / be. The vanillylmandelic acid (VMA), the homovanillic acid (HVA), or both, are elevated in more than ? 90% of patients. It can be used 24-h urine, but random urine is usually sufficient. If the primary site of neuroblastoma lies with the kidneys, it must be distinguished from Wilms tumor and other kidney tumors. It must also be distinguished from a rhabdomyosarcoma, hepatoblastoma, lymphoma and tumors genital origin. Staging of neuroblastoma, the following should be done to assess metastases: bone marrow aspirates and core biopsies from several places (usually from both posterior iliac crests) skeletal investigation bone scan or 131I-metaiodobenzylguanidine (MIBG) scan abdomen, and thorax Pelvis- computed tomography or magnetic resonance imaging When symptoms or findings suggestive of brain metastases, a skull computed tomography or -Magnetresonanztomographie should be. The results of these tests determine the degree (extent of spread) of the disease. The International Neuroblastoma Staging System (INSS) requires that the results of the operation to determine the degree. The International Neuroblastoma Risk Group Staging System (INRGSS) used by imaging-defined risk factors rather than surgery to classify neuroblastoma. Neuroblastoma has a unique stage, called 4S (per INSS) or MS (per INGRSS) which regresses often spontaneously without treatment. This phase involves children <12 months (4S) or 18 months (MS) where disclosure is restricted with localized primary tumor on the skin, liver and / or bone marrow. The involvement of the bone marrow should be minimized and limited to <10% of total nucleated cells and can not bark bone einbeziehen.Risikostratifizierung neuroblastomas At diagnosis should adequate attempts are being made to (suitable tumor tissue for analysis of DNA index the ratio of the amount of DNA in a tumor cell to the amount in a normal cell, the DNA index is therefore a quantitative measure for the chromosome content) and for amplification of MYCN oncogene to obtain. These factors help determine prognosis and guide the intensity of therapy. The categorization of risk is complex, and there are two large Stratifizierungssysteme for risk groups: one developed by the Children's Oncology Group (COG) and the other by INRGSS. These systems are based on the patient's age, stage, histology, MYCN amplification and DNA index. In the INRG chromosome 11q aberrations considered in the evaluation. In both systems, these factors are used to classify patients into low-, medium- and high-risk categories, which helps to determine the prognosis and forwards the intensity of the treatment. Prognosis The prognosis of neuroblastoma depends on age at diagnosis, stage and biological factors (eg. As histopathology, tumor cell ploidy in younger patients, MYCN amplification). Younger children with localized disease have the best prognosis. The survival rates for diseases with low and medium risk are about 90%. Historically, the survival rate is at high risk of disease in about 15%. This rate was improved to> 50% with the use of intensive therapy. And a recent randomized study showed that intensive therapy combined with immunotherapy to a 2-year event-free survival rate of 66% resulted. Treatment Surgical resection Normally chemotherapy Sometimes high-dose chemotherapy followed by stem cell transplantation Sometimes radiotherapy cis-retinoic acid for maintenance therapy in high-risk disease immunotherapy Treatment of neuroblastoma is based on the risk category (see also National Cancer Institute Overview of Neuroblastoma Treatment). Surgical resection is important for diseases with little un medium risk. She is often delayed until adjuvant chemotherapy is done to improve the opportunity for an appropriate surgical resection. Chemotherapy (vincristine, cyclophosphamide, doxorubicin, cisplatin, carboplatin, ifosfamide, and etoposide) is necessary in children whose disease has a medium risk. High-dose chemotherapy with stem cell and cis Retionins√§ure are often used in children with high-risk disease. Sometimes in children with moderate and high risk irradiation is necessary. An immunotherapy, which uses monoclonal antibodies to neuroblastoma antigens in combination with cytokines, is the latest approach to treatment of the disease with a high risk. More information International Neuroblastoma Staging System (INSS) International Neuroblastoma Risk Group Staging System (INRGSS) Children’s Oncology Group (COG Overview of Neuroblastoma Treatment (National Cancer Institute)

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