A neonate underwent MRI brain on post-natal day one, because of large head circumference at birth. The latest antenatal scan was maternal anomaly scan which did not reveal any abnormality.
A large midline space occupying lesion was seen in suprasellar region with cystic and solid areas. Areas of T2 hypointense signal with susceptibility were likely related to calcium deposition. The lesion exhibited mass effect on the ventricular system with resultant hydrocephalus. Owing to large size, origin of the lesion could not be accurately determined, however possibility of hypothalamic glioma or germinoma was suggested and urgent neurosurgery opinion was recommended.
The child went on to have surgery with partial resection of the tumour and ventricular shunting. The histopathology came back as craniopharyngioma. In spite of the surgery the child ended up with associated morbidity in the form of delayed milestones.
Congenital brain tumours are defined as tumours occurring in first 60 days of life. Teratomas are the most common [2,3]. Craniopharyngiomas (CP) are extremely rare and constitute 0.5-1.5% of paediatric brain tumours [1-3]. CP arise from remnants of craniopharyngeal duct from Rathke’s cleft to anywhere along third ventricle. Strictly speaking CP are suprasellar rather than sellar lesions, however if sizeable, it is difficult to identify the epicentre of the lesion and difficult to narrow down the differential, especially in absence of classic features.
CP is a WHO grade I intracranial neoplasm, although could be locally invasive. It is bimodal with one peak being in the first two decades which is histologically adamantinoma type and second peak occurs in 5-6th decades, which is papillary type. Although mixed types are also not unusual. No gender predilection .
The recent increase in the incidence of congenital CP is due to improved diagnostic tools. USG is often the first diagnostic tool. Either the lesion per se or indirect evidence like hydrocephalus and other associated findings are diagnosed antenatally. Some of them go on to have antenatal in-vivo MRI brain for further characterisation depending on the available resources and age of gestation.
Depending on the size of the lesion USG can detect echogenic solid components, cystic degeneration and calcific components. Real time vascularity can be assessed using Doppler which is also a useful tool to differentiate CP from other midline vascular anomalies. Associated hydrocephalus can be quantified and serially monitored. If there is increase in head circumference as a result, USG helps in monitoring head circumference and decision regarding mode of delivery can be made accordingly.
Although post-natal neurosonogram can assess the lesion, MRI is a better option.
Not a diagnostic tool. Can be used for follow-up, for monitoring post-therapy changes, ventriculomegaly and complications if any, especially in emergency settings. CT does identify the calcific components better than MRI.
In centres where routine antenatal maternal MRI is performed, additional findings can be found out apart from better soft tissue details. As most of the paediatric CP are adamantinomas which are prone to cystic degeneration, these can be accurately assessed. Antenatal MRI helps to plan the pregnancy outcome or determining the mode of delivery and aids in preparedness of neonatal team in terms of treatment planning.
Routinely postnatal MRI is performed in the first week after birth to compare the size to prenatal imaging thus assessing growth and also as a baseline for planning of interventions like surgery. If needed gadolinium can be administered which shows enhancement of solid components.
Treatment and prognosis:
If vaginal delivery is attempted, cystic component can rupture and result in chemical meningitis. Hence caesarean section is usually performed followed by post-natal MRI in view of planning surgery.
Prognosis depends on size of the tumour, extent of resectability (larger ones have poorer prognosis) as radiotherapy is contra-indicated in newborn . Prognosis is generally poor with increased morbidity even in surgically treated cases in terms of delayed milestones, visual disturbances due to optic chiasmal atrophy and learning disabilities.
Written informed patient consent for publication has been obtained.
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