Abstract
The pathological findings and clinical course of a case of primary cerebellar neuroblastoma was reported.
The patient, a two-month-old girl, showed sun-set phenomenon and bilateral foot-clonus. Retrobrachial angiography and CT scan showed hydrocephalus and a large tumor in the posterior fossa. Partial removal of the tumor and a ventriculo-peritoneal shunt were performed. Thereafter, radiotherapy was given (whole brain 2, 940 rads, total spinal 2, 000 rads), and methotrexate was injected intrathecally through the ventricular tube of the shunt every 6 months. On discharge, CT scan did not reveal tumor and 29 months after the operation the patient showed no signs of tumor recurrence on CT scan or in neurological examination. Histologically, the tumor was lobulated by narrow vascular stroma. The tumor cells had little cytoplasm, a round or oval nucleus, and were arranged in groups of variable sizes or in single rows, separated by varying amounts of fibrillary matrix. Ultrastructurally, the extracellular spaces were occupied by varying amounts of intricate networks of slender cytoplasmic processes which lay mostly parallel each other and contained many microtubules and clear vesicles of about 40-50 nm in diameter. These clear vesicles occasionally aggregated in groups and assumed a presynaptic configuration associated with a postsynaptic membrane thickening. These findings established the diagnosis of neuroblastoma. This case, as confirmed by the literature, indicates that the prognosis of cerebellar neuroblastoma is better than for medulloblastoma.
