Neuroblastoma - symptoms and signs, treatment
Neuroblastoma is a tumor that develops in the adrenal glands or less often in the adrenal gland sympathetic ganglia, including the retroperitoneal, ganglion of the thoracic cavity and neck. The diagnosis is based on the results of a biopsy. Treatment may include surgical resection, chemotherapy, radiation therapy and high-dose chemotherapy with stem cell transplantation.
Neuroblastoma is the most common tumor in children of the first years of life. Almost 90% of all cases occur in children under 5 years of age. There are a large number of various cytogenetic disorders in several chromosomes that can lead to the development of neuroblastoma; in 1-2% of cases, these anomalies are hereditary. Some markers correlate with tumor progression and prognosis.
In about 65% of cases, the primary tumor site is located in the abdominal cavity, in 15-25% - in the chest; in other cases, the primary localization of the tumor is the neck, small pelvis or other places. Neuroblastoma very rarely develops as a primary tumor of the CNS.
Most neuroblastomas produce catecholamines, which may be the presence of characteristic tumor cells during bone marrow puncture or trephine biopsy, in combination with an increased level of catecholamine metabolites in the urine. More than 90-95% of patients have an elevated level of vanilliginal acid, homovanilic acid, or both. You can use daily urine, although usually enough research single dose of urine. Neuroblastoma should be differentiated from Wilms tumor, other kidney tumors, rhabdomyosarcoma, hepatoblastoma, lymphoma and genital tumors.
To detect metastases, a bone marrow puncture and trephine-biopsy from different sites, a skeleton examination, bone scan or scintigraphy with metaiodobenzylgua-nidine, as well as CT or MRI of the abdominal cavity and chest should be performed. A CT scan or CT scan of the brain is indicated if symptoms or signs indicate a metastasis in the brain. When a tumor is resected, a part of it should be examined for the DNA index, and N-myc oncogene should be amplified in it to determine the prognosis and intensity of therapy.
Neuroblastoma - treatment
The prognosis is better in children with a low-risk tumor [age less than 1 year, lack of amplification of the N-myc oncogene, and the first stages of the tumor]. Chemotherapy, as a rule, is necessary in children with a medium-risk tumor. High-dose chemotherapy with stem cell transplantation and cis-retinoic acid is often used in children with a high-risk tumor. Radiation therapy is sometimes necessary in children with a medium to high risk tumor.
Found as increased urinary excretion of catecholamine metabolites. Ganglioneuroma is a completely differentiated, benign variant of neuroblastoma.
Approximately 40–50% of children at the time of diagnosis only have a primary focus or metastases in regional lymph nodes, 50–60% of children have distant metastases.
Neuroblastoma - Symptoms and Signs
Symptoms and signs depend on the localization of the primary tumor and its mode of growth and the path of metastasis. The most common symptoms are abdominal pain, discomfort and a feeling of fullness due to tumor masses in the abdominal cavity. In some children, the first manifestation is bone pain due to common metastases. In case of retrobulbar metastases, periorbital ecchymosis and proptosis may occur.Liver metastases, especially in infants, can cause abdominal enlargement and respiratory disorders. Sometimes pallor, petechiae and leukopenia develop in connection with metastases in the bone marrow. Other rare sites of metastasis include the skin and brain. Hemorrhage into the tumor and its necrosis may occur. Due to the direct spread of the tumor into the spinal canal, local children sometimes develop local neurological symptoms and paralysis. Manifestations of paraneoplastic syndromes, such as cerebellar ataxia, opsoclonus-myoclonus, watery diarrhea or hypertension, may also be noted.
Neuroblastoma - diagnosis
Routine prenatal ultrasound of the fetus sometimes reveals neuroblastoma. Patients with abdominal symptoms or an abdominal mass should be given an ultrasound or CT scan. The diagnosis is then confirmed using a biopsy of any detected lesion.
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