Congenital brain tumours are infrequent and account for approximately 1–4% of all paediatric cases.¹ One to four live births per 100,000 are estimated to be affected by a congenital brain tumour.^2,3 The majority of these neoplasms reside in the supratentorial compartment and, as noted by Volpe, the clinical manifestations of congenital brain tumours usually involve one or more of four typical syndromes.⁴ These syndromes are predicated on:

• macrocrania;
• hydrocephalus;
• focal neurologicsl deficit(s); and
• acute intracranial haemorrhage.

Although neuroimaging modalities are increasingly identifying cases during foetal development, aggressive surgical and chemo-/radiotherapeutic regimens have yet to significantly alter the dismal prognosis for most of these unfortunate patients.⁵ As determined by Isaacs in his 2002 review, overall survival is only 28%.^2,3

Numerous small case series and larger reviews have been published on the topic of early-onset paediatric brain tumours.^2,3,5–13 Ascertaining the frequency of individual congenital tumour types is often made difficult by the inclusion of older children in some analyses (up to 18 months of age in one study).^11–13 In particular, these latter studies often underestimate the frequency of teratomas. For example, in Larouche et al.’s review of 1,289 infants less than one year of age, astrocytomas accounted for 30.5% of the cases, while teratomas accounted for only 4.9% of the total.¹¹ Of the larger studies that were more strictly confined to the congenital time period, i.e. all cases less than or equal to two months of age, teratomas and astrocytomas were usually the most frequent tumour type.^2,3,5,8 In Isaac’s 2002 analysis of 225 congenital brain tumours, teratomas (44.9%) were by far the most frequent type, followed by astrocytomas (16%) and medulloblastomas (7.6%).^2,3 Buetow et al.’s 1990 review of 45 congenital cases included three main categories: 13 astrocytomas (nine grade I–III and four glioblastoma); 12 primitive neuroectodermal tumours (PNETs), four of which were medulloblastoma; and 12 teratomas.⁸ Finally, the study by Cassart et al. in 2008 of 27 foetal cases included 13 teratomas and four glial tumours (subtype unspecified) without any medulloblastomas/PNETs.⁵

To read full article please click here