Neonatal brain tumours — A review
Section snippets
Incidence
The incidence in the UK of central nervous system tumours in 2000 was approximately 2–3 per 100,000 children under 1 per annum, accounting for 14% of cancers diagnosed in the less than 1 year age group [1]. This compares to a rate of approx. 3 per 100,000 p.a. across Europe for CNS tumours in children up to the ages of 14 [2]. Overall in USA they account for < 5% of all newborn and stillborn children diagnosed with cancer. The term ‘central nervous system tumours’ (CNS) in these epidemiological
Presentation
The presentation of CNS tumours in the neonatal group is varied, and can be categorised into antenatal and perinatal/neonatal presentations. The near ubiquitous use of antenatal ultrasound scan by Western European/North American maternity services has meant brain tumours can be detected before birth. Features that may suggest the presence of a brain tumour include intracranial mass, abnormal echogenicity in the head, macrocephaly and hydrocephalus, although it should be stressed that the
Investigation
The mainstay of the investigation in children with suspected tumours is magnetic resonance imaging (Fig. 1). Imaging of the entire neuraxis is indicated as germ cell tumours, PNET tumours and occasionally pilocytic (benign) lesions have a propensity to metastasise throughout the subarachoid space [8] (Fig. 2). MRI has the advantage of high resolution imaging of soft tissues allowing for detailed assessment of tumour morphology and relationship to surrounding structures. For certain types of
Long-term outcome
The most common outcome of a CNS tumour in neonatal patients is death within 5 years of diagnosis. However, the survivors are likely to suffer long-term morbidity. The cumulative mortality of children who survive to 30 years is approx 25% [20]. The majority of these die from recurrent disease or new CNS neoplasm, the risk of which is related to exposure to adjuvant irradiation. Survivors are more likely to suffer from endocrine, neurological or sensory symptoms than siblings, and more likely to
Key guidelines
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The principle guideline for those involved in the care of foetuses/neonates with primary CNS tumours is to obtain informed consent from the parent(s) of afflicted children before contemplating further management. Parents should be counselled about the poor prognosis of many forms of tumour and morbidity associated with treatment should be explained to the parents. However, ‘cure’ is possible with some forms of tumour and it should be explained to the parents that it may not be apparent until
Research directions
To identify the causative mechanisms especially the role of environmental factors such as exposure to non-ionising radiation sources e.g., radiofrequency, radio waves and microwaves, during pregnancy.
Parental exposure to smoking and viral infections during pregnancy as a link to cause needs further research.
Acknowledgements
The author would like to thank Mr Martin Gilles for the valuable assistance in the preparation of this article, and Mr P G Richards and Mr J. Jayamohan for their editorial assistance.
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