Cerebral ganglioneuroblastoma of adult onset: two patients and a review of the literature

Abstract

Ganglioneuroblastoma is a rare tumor variant of neuroblastoma. Only five cases have been observed in the adult brain, and we report here on two more adult patients with cerebral ganglioneuroblastoma. Additionally, a review was carried out on all 50 published adult cases with ganglioneuroblastoma, located in the adrenal gland (9), mediastinum (8), retroperitoneal area (7), the brain parenchyma (7), or the spinal cord (3). Median age at onset was 39 years, and 52% of patients were female. For extracranial locations, treatment usually consisted of surgery followed by radiotherapy and adjuvant chemotherapy. Of the cases with cerebral involvement only one patient did not receive any treatment. The other six patients underwent surgical resection and radiation therapy, in four cases followed by chemotherapy with temozolomide. The median survival of cerebral ganglioneuroblastomas was 14 months and did not differ from the whole group of ganglioneuroblastomas (12 months). For cerebral ganglioneuroblastoma, the preferred regimen would seem to be neurosurgical removal, followed by chemoradiotherapy including temozolomide.