Congenital medulloblastoma

A 14 day old baby girl was referred to the neonatal intensive care unit with poor feeding, respiratory distress, drowsiness, vomiting, and hypoactivity of one day duration. She was born by caesarean section to a 21 year old mother, gravida 1, para 1, after an uneventful pregnancy. The baby appeared normal at birth, and Apgar scores were 9 and 10 at one and five minutes respectively. On physical examination, the baby was lethargic, hypotonic, and macrocephalic, with no suck or Moro reflex. Her anterior fontanelle measured 4 cm and was bulging. Other systems were normal. Magnetic resonance imaging revealed a mass with heterogenic intensity in the posterior fossa (fig 1). In T2A series, the mass had hyperintense cystic components and fibrous septations. The patient was operated on the following day. The pathology was consistent with a medulloblastoma. On histochemical examination tumour cells were positive with synaptophisin and vimentin, focal positive with S 100.

Figure 1 Magnetic resonance imaging scan showing a mass with heterogenic intensity in the posterior fossa. Permission for publication of this figure has been given by the patient's parents.

Neonatal brain tumours are rare, accounting for about 1% of all paediatric tumours. Among these, neonatal medulloblastoma is extremely rare.1,2,3 Medulloblastoma occurs only in the posterior fossa, although it has been grouped with histologically similar tumours arising elsewhere as a primitive neuroectodermal tumour.