What about central neuroblastoma?

Patient Cheng Moumou, female, 66 years old, complained of loss of vision in both eyes for six months, headache and unresponsiveness for three months. On preoperative examination, visual acuity in both eyes was 0.4 in the left eye and 0.8 in the right eye, and there were no obvious positive signs in the nervous system. Head MRI showed irregular nodular mixed signal shadow 3CM×4CM×3CM in the suprasellar pool, the lesion protruded into the dorsum of the saddle, the adjacent brainstem was obviously compressed, the visual crossover was not clear, the left cavernous sinus was obviously involved, and the enhancement scan showed obvious irregular nodular enhancement shadow in the saddle. Preoperatively, pituitary tumor was considered, and craniopharyngioma was not excluded. Under general anesthesia, left lateral pterygoid approach via lateral fissure brain tumor resection was performed, during the operation, the tumor in the anterior part of the third ventricle was firstly revealed, which was fleshy red, soft, with general blood supply, and the left optic nerve was extruded and deviated to the right side, and invaded the left internal carotid artery, anterior cerebral artery and anterior communicating artery with partial encapsulation, which was resected in parts, and a little remnant was found at the wall of left internal carotid artery, and intraoperative freezing was considered as a CNS neuroblastoma. The same method was used to treat the tumor in the third ventricle and the saddle region, and to open the cerebrospinal fluid circulation. Postoperative pathology report: central neuroblastoma. He was recommended to receive local radiotherapy outside the hospital, and was followed up for 1 year and 3 months, with no significant changes in the residual tumor. Discussion Central neuroblastoma (CN) was first reported by Hassoun in 1982, and since then the disease has attracted much attention. It is generally regarded as a benign tumor with a good prognosis, but in rare cases, it may become malignant and recur. The most common age is between 15 and 52 years old, and it is more common in young and middle-aged people. The tumor grows slowly and has a long course. It is generally believed that the tumor mainly occurs in the anterior wall of the lateral ventricle, mostly adjacent to or originating from the pars compacta, and six cases of CNS tumors occurring in the cervical medulla have been reported in foreign literature. In this case, it was rare to find a tumor with saddle region occupancy and optic nerve symptoms as the main complaint, and it was easy to be misdiagnosed preoperatively. Its imaging characteristics: atypical CT manifestation, it is an isohyperdense mass connected with the septum lucidum with uneven isosignal in T1WI and relative isosignal in T2WI, and the degree of enhancement after enhancement is not the same, which is a typical “bubble-like” change. When the tumor is widely adherent to the lateral wall of the ventricle or the septum pellucidum, the disease should be highly suspected. In this case, irregular nodular mixed signal shadow was seen in the suprasellar pool, and the enhancement scan showed irregular nodular enhancement, which was one of the reasons why the diagnosis was not made clearly before surgery. Surgical resection is the best means to treat this disease. The purpose of surgery is to resect the tumor, open the cerebrospinal fluid circulation pathway, and relieve intracranial hypertension. Surgical methods include frontal cortical anterior horn fistula and longitudinal fissure approach. In this case, because the tumor was located in the saddle region, and the invasion to the left cavernous sinus was obvious, and the damage to the left optic nerve was obvious, the left lateral pterygoid point of approach was used for resection through the lateral fissure. For those with rapid tumor growth, obvious infiltration of surrounding tissues, a large number of mitotic signs, cell proliferation and necrosis found under light microscopy, and those who have not been completely resected, local radiotherapy should be given after surgery, and the long-term efficacy is ideal and long-term survival can be achieved.