Type:
Educational Exhibit
Keywords:
Neoplasia, Contrast agent-intravenous, MR, CT, Paediatric, Oncology, Head and neck
Authors:
E. Garcia Martinez1, D. H. Jiménez1, L. Navarro Vilar2, C. P. Fernandez Ruiz1, M. Atares 3, F. Menor1; 1VALENCIA/ES, 2Valencia, Va/ES, 3Picaña, Valencia/ES
DOI:
10.1594/ecr2013/C-2220
Background
Brainstem diffuse gliomas have a poor prognosis and is important to recognize their image findings.
These tumors are infiltrating astrocitomas,
and most frequently arise in the pons,
typically present in childhood (3 to 10 years of age) and make up 10 - 15% of all paedriatric brain tumors and 20 - 30% of paedriatric posterior fossa tumors.
Typically patients present with multiple cranial nerve palsies and signs of raised intracranial pressure.Cerebellar signs may also be elicited including ataxia,
dysarthria,
nystagmus and sleep apnoea.
These tumours can be divided by location in mesencephalic,
pontine( most common 60 - 75% of all cases) or medullary.
There is an association with neurofibromatosis type I,
which however carries a better prognosis with a more indolent course.
Due to the high rate of severe complications with biopsy treatment is usually commenced without histological confirmation.
Treatment usually comprises of chemotherapy only.
In children over 3 years of age (or preferably even older) then radiotherapy may be considered.
Necrosis may result from radiotherapy,
however it may also be seen as part of the natural course of the tumour.
Initial response can be dramatic and falsely reassuring.In the sporadic form the prognosis is poor with 2 year survival being only 20% (median survival less than 1 year).