A case of primary cerebral neuroblastoma was reported with respect to the clinical course and pathological findings. The patient, a three-year-old girl, suffered from headache and vomiting. Carotid angiography and CT scan showed a large tumor in the right parieto-occipital lobe expanding to the right lateral ventricle and crossing the midline to the opposite side. A total resection of the tumor was performed, and Linac therapy with a tumor dose of 5, 100 rads and chemoimmunotherapy (Me-CCNU, vincristine, OK-432) were given. Repeated CT scan showed recurrence of the tumor three months after surgery, and further irradiation therapy (tumor dose of 4, 500 rads) was given. She continued to deteriorate with multiple pulmonary metastases, and died eleven months after the initial symptoms and nine months after the surgery.
Histologically the tumor cells were small, uniform and spherical, were arranged in delicate fibrillary stroma, and occasionally formed Homer-Wright rosettes. Electron microscopy showed neuronal processes with growth cones and rosettes with distinct lumina. The luminar surfaces were covered with a smooth plasma membrane lacking any surface differentiation and the lateral surface of these cells had many cell junctions, reminiscent of a primitive neural tube.