Ganglioneyroblastoma - causes, symptoms, diagnosis and treatment
Ganglioneyroblastoma - Malignant neurogenic tumors containing neuroblasts and at different stages of differentiation ganglion cells. Ganglioneyroblastoma is characterized by anorexia, weight loss, fever, symptoms of catecholamine excess (increased blood pressure, sweating, facial flushing, tachycardia). Depending on the location of the tumor can be observed symptoms of compression of the abdominal cavity or mediastinum, focal neurological symptoms. Diagnosis is based on ganglioneyroblastomy tumor imaging with ultrasound, CT, MRI, MSCT and its morphological verification of the study of biopsy material. The most effective way to treat ganglioneyroblastomy - is radical or extended removal of the tumor.
Ganglioneyroblastoma
ganglioneuroma and occupies an intermediate position between that of benign tumor and highly malignant neuroblastoma. Today, neuroscience and medicine in general, do not have precise information about the reasons that lead to the development of ganglioneyroblastomy. Ganglioneyroblastoma most often found in children, mostly in the age range from 4 to 10 years. Separately emit so-called combined ganglioneyroblastomu having ganglioneuroma structure with areas of neuroblasts. This ganglioneyroblastoma found mainly among patients older than 10 years and the average age of onset is 20 years.
anorexia, anemia, fever. There may be periorbital edema. Since ganglioneyroblastoma accompanied by an increase in blood koteholaminov (adrenaline, dopamine, norepinephrine), clinically often observed due to their excess symptoms: sweating, hypertension, tachycardia, facial flushing, sometimes - chronic diarrhea.
Ganglioneyroblastoma mediastinum is characterized first and foremost a violation of the respiratory function: shortness of breath, cough, obstructive breathing or stridor, apnea attacks. These symptoms are more often caused by reflex mechanisms of neuropathic and paresis of the larynx tumor arising in the compression of the vagus nerve or its branches. They are characterized by the sudden appearance and rapid development in the clinic for several days. In this case the tumor may have a small size, does not cause displacement of the mediastinal structures and spread to the lungs. If ganglioneyroblastoma puts pressure on the thoracic sympathetic nodes, the clinical picture is observed violation of sweating, anisocoria (different size pupils), Horner's symptom: upper eyelid ptosis (ptosis), mydriasis (cramps), enophthalmos.
brain Ganglioneyroblastoma has a clinical picture of intracerebral tumor with focal symptoms, appropriate to its location. Usually, it spreads within the central nervous system. Ganglioneyroblastoma extracerebral localization metastasizes to the lymph nodes, bones, liver and other internal organs.
catecholamines in blood and their metabolites (vanillylmandelic acid and homovanillic acid) in urine.
X-rays can only provide indirect evidence of tumor. With its location in the retroperitoneal space to survey radiography of abdominal organs revealed stretching bowel loops and paravertebral calcification, at the location of the tumor in the mediastinum on chest radiograph bodies can reveal a rounded dense shadow, the local expansion of intercostal spaces, the presence of "Uzury" on the edges in places their contiguity to the tumor.
In order to visualize ganglioneyroblastomy used MSCT or ultrasound of the abdomen, CT of the chest, pnevmomediastinografiya, MRI of the brain. For the detection of tumor metastases is applied radiography and scintigraphy of the skeleton, liver ultrasound, needle biopsy of a lymph node, etc.
Finally ganglioneyroblastoma can only be diagnosed by microscopic examination of the tumor sample obtained intraoperatively or during biopsy. In the diagnosis of brain ganglioneyroblastomy stereotactic biopsy can be used. Histologically, in the case of the drug found in ganglioneyroblastomy small immature neurocytes with varying degrees of differentiation, large multi neurocytes with vacuoles in the cytoplasm, areas of necrosis and small cysts. Combined ganglioneyroblastoma characterized ganglioneuroma similar to the structure, the distinguishing feature is the presence of lesions composed of neuroblasts, which may or may not have signs of differentiation.
Ganglioneyroblastoma requires differentiation of other malignancies that have small cell structure, for example, lymphoma, glioblastoma and metastatic tumors.
neurosurgeons in accordance with the principles of operations in brain tumors. Surgery at ganglioneyroblastome mediastinal and retroperitoneal produce surgeons and thoracic surgeons. At the same time, if ganglioneyroblastoma not grow into the surrounding tissue, it made its radical removal. However, most have to perform the extended removal of the tumor, including resection of the adipose tissue and regional lymph nodes. In cases where ganglioneyroblastoma can not be completely removed for decompression of the surrounding anatomical structures produce palliative tumor removal, ie. E. Its partial resection.
Surgical treatment ganglioneyroblastomy combined with chemotherapy drugs vincristine and cyclophosphamide. In older children may use radiation therapy. In severe cases may require a bone marrow transplant.
Forecast ganglioneyroblastomy
The average 2-year survival of patients with ganglioneyroblastomoy is 70%. As shown by clinical observations, ganglioneyroblastoma localized in the posterior mediastinum, has a favorable prognosis. When her 2-year survival rate approaching 80%, whereas in retroperitoneal localization ganglioneyroblastome it stands at 60%. Patients nonproliferation ganglioneyroblastomoy and after surgery live longer than 2 years more than 95% of cases. With widespread nature of the tumor and the presence of metastases 2-year survival rate is only 50%.