central neurocytoma
Introduction
Introduction to central neurocytoma Central neuroblastoma is a small cell neuron tumor that grows in the lateral ventricle and the third ventricle. Its main site is in the transparent interventricular septum (Monro hole). When the clinical symptoms are caused, the tumor grows very large. The main symptoms are symptoms of increased intracranial pressure caused by headache and obstructive hydrocephalus. basic knowledge The proportion of illness: 0.003% Susceptible people: no specific population Mode of infection: non-infectious Complications: cerebral edema meningitis
Cause
Cause of central neurocytoma
(1) Causes of the disease
In 1982, Hassoun et al first discovered the specificity of its ultrastructure, which is considered to be the origin of nerve cells, but it is different from ganglioneuroma and neuroblastoma under light microscope. The intraventricular nerve cells may come from transparent compartment. Or granule neurons of the small gray matter nucleus.
(two) pathogenesis
Central neuroblastoma overexpresses embryonic neural cell adhesion molecules, but lacks neurofilament proteins and mature synapses, so its occurrence may be due to abnormal gene expression in embryonic neurons and lack of further differentiation.
In terms of pathology, the main part of the tumor is located in the lateral ventricle, the boundary is clear, and some of the lateral ventricle wall is attached. It can originate from the transparent sputum and the corpus callosum. It can also adhere to the frontal lobes or erode the frontal lobes. The midbrain aqueduct or the fourth ventricle, the tumor obstructs the interventricular orifice, the third ventricle or the midbrain aqueduct can cause the ventricle to enlarge, produce obstructive hydrocephalus, the tumor becomes spherical, the boundary is clear, the tumor texture is soft, gray Red, with calcification.
The morphology of tumor cells under light microscope is very similar to that of oligodendroglioma. It is difficult to distinguish. It consists of a single small cell, with little quality, round nuclear, chromatin is spotted, often with nuclear perihelosis, localized calcification in the tumor. In the tumor, some tumors contain a perivascular pseudo rosette-like structure resembling an ependymoma. If there is a mitotic image, tumor necrosis or vascular endothelial proliferation, it suggests tumor malignancy. By immunohistochemistry, it can be used for central neurocytoma and less gum. Differentiation of stromal tumors and ependymoma, synaptophysin is strongly positive in most central neuroblastomas, while glial fibrillary acidic protein (GFAP) is negative, in addition, some tumor neuron-specific enolases ( NSE) staining positive, for a small number of synaptophysin-negative tumors, the diagnosis depends on electron microscopy to observe the ultrastructure of cells. Under electron microscope, there are a lot of neurosecretory granules, synapses, microtubules and axes in the cytoplasm of tumor cells. A neuron-like structure.
Prevention
Central neuroblastoma prevention
Without special precautions, most of the central nervous cell tumors have benign biological behavior, and most of the prognosis is good. Because the tumor has the possibility of infiltration and growth at the attachment of the ventricular wall, the complete operation of the tumor alone can not prevent tumor recurrence. Studies have reported that patients with central neurocytoma who have undergone surgery alone have recurred within 3 years, and combined with postoperative radiotherapy, even if the tumor is partially resected, most patients can get long-term cure. Another study summarized 20 cases of central nervous cells. Except for 3 cases of surgical death, the longest follow-up period was about 19 years. The domestic authors reported 13 cases of lateral ventricle neuroblastoma. Among them, 6 cases of postoperative radiotherapy patients had no tumor recurrence after 1 to 8 years of follow-up. One of them only underwent biopsy during operation. The tumor disappeared completely after radiotherapy. There was no tumor recurrence, and the other patient did not receive radiotherapy after surgery, and the tumor recurred after 16 months. The 5-year survival rate of general central neuroblastoma is 81%, and the 5-year survival rate of the whole cut can reach 90%. Radiotherapy is effective for subtotal resection and can prolong survival.
Complication
Central neurocytoma complications Complications cerebral edema meningitis
Concurrent obstructive hydrocephalus causes an increase in intracranial pressure. If surgery is performed, the following complications may occur: cerebral edema, postoperative intracerebral hematoma, neurological dysfunction, and meningitis.
Symptom
Symptoms of central neurocytoma Common symptoms Responsive, strong grip, reflex sensation, hydrocephalus, nausea
Central nervous system tumors are more common in young and middle-aged people. The age of onset is generally 15 to 52 years old. The average age of onset is about 30 years old. The ratio of male to female is almost equal. The ratio of male to female is 11:9. The ratio of male to female is 0.86. 1, the average duration of central neurocytoma is 3 to 7 months, because the tumor is located near the Monro hole, the clinical manifestations of the symptoms of increased intracranial pressure caused by obstructive hydrocephalus, because the tumor is located in the lateral ventricle, the patient's main performance For the symptoms of increased intracranial pressure, the clinical symptoms are not obvious at the beginning of the disease, a few patients have mild headache or discomfort, dizziness, dizziness, headaches increase with tumor growth, headaches are frequent, duration increases, when tumor growth blocks interventricular pores or When entering the third ventricle to block the midbrain aqueduct, the patient turned into persistent headache, nausea, frequent vomiting, unclear vision, and even blindness. Some patients were unresponsive due to tumor involvement in the frontal lobe, groping, strong grip reflex Positive equal frontal lobe symptoms may have olfactory abnormalities or olfactory loss and illusory scent. When the tumor is located in the triangle of the lateral ventricle, some patients may be biased. Or partial sensory disturbance, there are reports of subarachnoid hemorrhage or amenorrhea caused by tumor stroke, most patients have no local signs, the most common signs are optic disc edema, in addition to hemiparesis, partial sensory disturbance and pathological signs positive .
Examine
Central neurocytoma examination
CT scan showed that the tumor was located in one side of the ventricle or in the transparent sputum. The boundary in the ventricle was clear, and the circular isometric density or slightly high and uneven density. More than half of the tumors were a little calcified. When the calcification was large, the X-ray was flat. It can also be seen that after the enhancement of the supratentorial central neurocytoma, the tumor has moderate to obvious enhancement. MRI imaging is superior to CT, and has diagnostic value for the tumor range and location. Most tumors are related to the transparent septum or lateral ventricle wall. T1W in the parenchymal part of the tumor is equal or slightly higher signal, T2W is a high signal, and vascular flow is visible in the tumor. Some tumors are often accompanied by hemorrhage. After intravenous injection of paramagnetic developer, mildly enhanced tumor and lateral ventricle wall or The transparent enamel is attached.
Diagnosis
Diagnosis and diagnosis of central neurocytoma
Lateral ventricle tumors with increased intracranial pressure in young and middle-aged patients, especially CT scans or MRI images of the skull, should be considered in patients with atrial calcification, and postoperative light microscopy is difficult to be associated with less glial The difference in cell tumors, the exact histological diagnosis depends on transmission electron microscopy or immunohistochemistry to show specific neuronal antigenic examination.
The disease should be differentiated from lateral glomerular glioma, meningioma, ependymoma, subependymal astrocytoma and astrocytoma. These tumors occur in the lateral ventricle. Clinical symptoms and signs are difficult to distinguish from lateral ventricle neuroblastoma, but imaging suggests that the tumor is located on one side or bilateral transparent septa, growing to other parts of the ventricle, and the tumor shows punctate or platelet calcification. According to the literature, it is difficult to distinguish neuroblastoma from the age of onset, gender, and pathological examination of oligodendrocyte tumors in the ventricle. The diagnosis is mainly based on electron microscopy or immunity. Histochemical examination.
