Occipital ganglio-neuroblastoma in an adult

Ganglioneuroblastomas (GNBs) are embryonic neoplasms, whose behaviour are not well established; 80% of cases occur in the first decade, while only two cases in the adulthood had been reported.

This 60-year-old female presented with a 2-month history of headache, vertigo, amnesia. A right brachio-crural hemiparesis, right homonymous hemianopsia and sensorial dysphasia was evident. A CT scan revealed a left occipital lesion. MRI scan was not performed because the patient had an anal sphincter stimulator. Three years before thyroidectomy for a follicular carcinoma was performed. A total body CT scan was negative. A left occipital craniotomy was carried out and a solid mass was totally excised. A diagnosis of GNB was made from histopathological examination and immunohistochemistry. Post-operatively the patient recovered from the hemiparesis while the visual disturbances persisted. She underwent fractioned radiotherapy (60 Gy) and chemotherapy with Temozolomide. She remained disease free 18 months after diagnosis. A CT scan showed no evidence of recurrence.

Recent observations suggest that in spite of an embryonal appearance, these tumours are circumscribed and have a better prognosis than malignant gliomas.