Introduction

Neoplasm developing in the intrauterine period and detected either at birth or in the first month of life represents 2.5% of all tumours in the paediatric age group. Congenital intracranial tumours are even more rare and only account for 0.5% to 1.5% of all childhood brain tumours. The most common of these present at birth are teratomas.

Case report

A term baby was born by Caesarean section for failure to progress in labour following an uncomplicated pregnancy to a primigravida mother. Antenatal scans were normal. A 3.4 kg (50th centile) baby girl was born requiring no resuscitation. However, the baby had frontal bossing, a large anterior fontanelle and widely separated sutures with a head circumference of 39.7 cm (>99.6th centile). A neurological examination showed mild generalised hypotonia but was otherwise normal and she was able to begin establishing oral feeds.

A cranial ultrasound scan (Figure 1) showed hydrocephalus and an urgent CT brain showed marked ventriculomegaly with associated white matter change and calcification scattered throughout the cerebellum and occipital lobe. MRI angiography of the brain (Figure 2) confirmed a very large heterogeneous posterior fossa tumour causing massive obstructive hydrocephalus supplied by multiple blood vessels. There was no evidence of metastases elsewhere.

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Figure 1

CT scan.

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Figure 2

MRI scan.

Following lengthy discussions with her parents, it was agreed to proceed with resection given the baby’s good current neurological condition. Embolisation of several vessels was carried out prior to tumour resection but despite this there was significant bleeding leading to cardiac arrest and death. Tumour histology confirmed an immature teratoma not likely to be due to an inherited predisposition.

Discussion

Congenital intracranial tumours are rare, with teratomas occurring most commonly1 and accounting for 29% to 50% of central nervous system (CNS) tumours.2 Teratomas of the brain are thought to arise from the pineal gland and appear to involve the third ventricle resulting in obstructive hydrocephalus. Pathological enlargement of the head is the most typical initial sign of perinatal intracranial tumours. They are usually sporadic with some cases diagnosed antenatally.3 Outcomes are generally poor although resection, if possible, may be curative.