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Abstract: The patient presented with progressive headache with loss of vision, along with imaging suggestive of an occupancy in the interventricular foramen area, and was diagnosed with a central ganglioneuroblastoma. After aggressive treatment, the patient’s condition was significantly controlled. The main clinical symptoms of central ganglioneuroblastoma are related to the local occupying effect caused by the tumor, or the symptoms of chronic cranial hypertension caused by ventricular obstruction.
Basic information】Male, 23 years old
Disease Type】Central ganglioneuroblastoma
Hospital】Shanghai First People’s Hospital
Date of consultation】September 17, 2021
Treatment plan】Medication (injectable methylprednisolone sodium succinate + compound mannitol injection + glycerol fructose sodium chloride injection) + tumor resection + lateral ventricular external drainage + ventriculo-abdominal shunt
Treatment period】17 days of hospitalization, 3-6 months of follow-up review
Treatment effect】The condition was significantly controlled and all indicators were improved.
I. Initial consultation
The patient complained of a dull painful headache and decreased visual acuity, and went to an external ophthalmology clinic for bilateral optic papilledema. At that time, according to the patient’s symptoms, it was more in line with the typical symptoms of chronic cranial hypertension, and the cranial MRI scan carried by the patient suggested that there was a round-like iso-low signal lesion in the right ventricular foramen area, combined with the manifestation of supratentorial ventricular enlargement.
II. Treatment process
After admission, the patient underwent further examination, including enhanced MRI, which suggested significant occupancy enhancement and rich blood supply. The patient was then given hormone therapy with injectable sodium methylprednisolone succinate. 3 days later, a repeat cranial CT did not show any reduction in tumor size, so the possibility of lymphoma was initially excluded. In addition, germ cell tumor was also found in the midline area. Therefore, β-HCG and methemoglobin in the blood and cerebrospinal fluid were tested, which were both negative, and the possibility of germ cell tumor was initially excluded. The patient was given compound mannitol injection combined with glycerol fructose sodium chloride injection to lower the intracranial pressure, and the symptoms were relieved. After completing the routine preoperative examination, the family was informed that the decision to perform total resection of the tumor would be based on the pathological results of intraoperative cryopreservation, and the family expressed that this plan was reasonable and requested to minimize the surgical damage to the patient. The intraoperative pathology suggested that the patient had a central neuroblastoma, so the tumor was resected under the microscope, i.e., the tumor was completely removed using the Endport technique combined with intraoperative navigation, and to prevent postoperative hydrocephalus, an external drainage of the lateral ventricle was performed at the same time. The patient’s headache was significantly relieved after the operation, and his vision was slightly better than before. With the improvement of the laboratory results, the ventricular drainage of cerebrospinal fluid gradually changed from light blood to colorless and clear, the ventricular drainage tube was removed in the first stage and the ventriculo-abdominal shunt was performed in the second stage in order to completely prevent hydrocephalus. The postoperative gross pathology reported that the central ganglioneuroblastoma was classified as WHO grade I.
III. Treatment results
Postoperatively, the patient’s headache symptoms were significantly relieved, blurred vision was improved compared with before. On imaging, there was no obvious blood leakage from the operated area, no abnormal cerebrospinal fluid laboratory tests, no intracranial infection, the ventriculo-peritoneal shunt was unobstructed in place, the size of the ventricle was reduced compared with before, and hydrocephalus was significantly improved compared with before. Because of the total resection of the tumor and the low malignancy of the tumor, there is no need for radiotherapy after the operation, and it is recommended to follow up with brain MRI in 3-6 months.
IV. Notes
We are glad that the patient’s headache and blurred vision have improved after treatment, but the patient needs to review the cranial enhancement MRI every 3-6 months after discharge to observe whether the tumor has recurred, and to observe the changes of hydrocephalus and ventricles. Secondly, patients need to press the shunt pump 50 times each morning, noon and night to prevent the shunt tube from blockage. Monitor the temperature closely. If the temperature exceeds 37.5℃ for 3 consecutive days, the patient needs to come to the hospital to identify the cause of fever. If the patient shows symptoms of ventricular enlargement again or intracranial hypertension, he/she needs to come to the hospital at any time.
V. Personal insight
Central ganglioneuroblastoma usually occurs in people under 30 years of age, which is consistent with the age of the patient in this case. Although central ganglioneuroma is relatively rare among intracranial tumors, it is important to consider the possibility of central ganglioneuroma when the tumor is located near the interventricular foramen in clinical practice, which usually presents with symptoms of chronic cranial hypertension and corresponding dysfunction and abnormal signs caused by local occupational effects. When the tumor is located in the supratentorial lobe, it can also cause epileptic symptoms, and when the tumor is located in the cerebellum, it can cause symptoms such as dysarthria, ataxia, and balance disorders. In terms of treatment, central ganglioneuroblastoma is not sensitive to radiotherapy and relies mainly on total surgical excision. When the tumor is located in the ventricular system, surgery is also required to release the obstruction of cerebrospinal fluid circulation. In terms of nursing care, since surgery in these patients often requires opening the ventricular system, aseptic care of the wound is crucial. generally, the duration of the external ventricular drainage tube should not exceed 7 days, and the daily drainage flow should not exceed 300 ml, with a height of 15 cm above the line of the external auditory canal on both sides. the surgical incision needs to be changed regularly to prevent the formation of cerebrospinal fluid leakage and subcutaneous fluid.