Background: Although rare, brain tumours represent one of the relatively larger groups of congenital neoplasias. Most studies on congenital neoplastic disease deal with several types of neoplasms and are dominated by leukaemias, retinoblastomas and systemic solid tumours. Few studies are dedicated to congenital brain tumours. We present nine newborns (four boys and five girls) who were diagnosed with congenital brain tumours during the 8-year period 1 January 1992-31 December 1999 at our institution, which covers all paediatric neuro-oncology cases for Eastern Denmark.
Epidemiology: Two of the cases were referred from Western Denmark for surgery, and were therefore excluded from the calculation of incidence. During the same period, a total of 172 children below the age of 15 years were diagnosed as having primary central nervous system tumours. The seven remaining congenital cases thus represent 4% of all paediatric brain tumour cases in the area (95% confidence interval 1.7-8.3%). The population of the referral area is 2.383x10(6), and based on the total number of living births, the incidence of congenital brain tumour was calculated to be 2.9 per 100,000 live births. The ages of the mothers were 28-33 years, corresponding to the present mean age of 31 years for Danish primipara. The gestational age varied between 35 and 42 weeks, and the birth weights were 3,044-4,790 g.
Risk factors: Two patients with p53-related glioblastoma multiforme (GBM) had relatives with p53-related neoplasms. In one case, the mother was treated for cancer of the ovary with surgery and chemotherapy 2 months before conception.
Clinical features: In five of the cases, brain abnormality was suspected antenatally. The clinical features of the newborns were limited to enlarged head circumferences, associated hydrocephalus, and asymmetric skull growth.
Diagnosis and treatment: Three babies were treated with complete tumour resection. In the remaining six cases, a guided or open biopsy to obtain histology was made after CT/MRI imaging. The histological diagnoses were teratoma in four cases, GBM in two cases, anaplastic astrocytoma in two cases and, finally, haemangioma capillare in one case.
Outcome: Four of the patients (44%) are still alive, including two patients with totally resected combined orbital/intracranial teratomas, one patient with a totally resected haemangioma and one patient with anaplastic astrocytoma who did not receive any treatment apart from supportive care. The survival lengths of the five neonates who died varied between 1 day and 51 days.