On May 16th the jounal Pediatric Radiology published an unsual case report of a a pre-natal DIPG written from the department of neurosurgery and diagnostic imaging t Brown University.
In this situation a 33 year female who had previously delivered 3 children without complications had an ultrasound at 33 weeks gestation. Unfortunately the ultrasound revealed a markedly enlarged head of a size more than 40 weeks but with an abdominal circumference and femur length of 33 weeks. There was also triventricular hydrocephalus without evidence of spina bifida.
A fetal MRI was then obtrained which showed a mass with the epicenter in the pons extending into the midbrain, medulla and cerebellar peduncles. The expansile mass had poor margins. There was severe hydrocephalus. No abnormalities were seen outside the brain. A diagnosis of diffuse pontine glioma was made.
The chid was delivered at 36 weeks, 4 days becaue of the big head and had Apgar scores of 7 and 9. A repeat MRI was preformed and confirmed the prior findings. The boy underwent placement of an extraventricular drain because of signs of increased intracranial pressure. The child died from respiratory failure at day 3.
At autospy the lesions was found to be primarily an infiltrative anaplastic oliogdendroglioma with areas of astroctyoma grade IV differentiation.
Although I have posted on neonatal tumors in the past (click here for prior blog post), the rarity of brainstem lesions in the neonatal period makes it difficult to know exactly what to do. Ther have been a few cases that have done well in the very young. There are other resports of the typically horrendous course of DIPG in the neonatal period. As researchers attempt to understand more about DIPG, this case adds to the library of knowledge regarding DIPG. The authors sugggest that autopsy studies should be strongly encouraged to learn more about this atypical situation. I would add that perhaps including this in the DIPG registry would also be helpful.
Reference-
Prenatal MRI characterization of brainstem glioma
in Pediatric Radiology
http://www.ncbi.nlm.nih.gov/pubmed/23677423
Spontaneous regression of a diffuse brainstem lesion in the neonate. Report of two cases and review of the literature
http://www.ncbi.nlm.nih.gov/pubmed/16206736
Lesion Regression
http://www.ncbi.nlm.nih.gov/pubmed/?term=AIREWELE+brainstem
Diffuse intrinsic brainstem tumors in neonates. Report of two case.
http://www.ncbi.nlm.nih.gov/pubmed/18447673
