Diffuse Intrinsic Pontine Glioma, Brain stem tumor скачать в хорошем качестве

Diffuse Intrinsic Pontine Glioma, Brain stem tumor

Dr Chor Ath Diffuse intrinsic pontine glioma Background Brain tumors are the most common solid pediatric tumor. Brainstem gliomas are relatively common pediatric central nervous system (CNS) tumors, representing approximately 15% of primary CNS tumors in children. The pons is the most common location for brainstem gliomas. It accounts for approximately 80% of cases. Patients with a diffuse intrinsic pontine glioma have a very poor prognosis. Although only comprising 15% of primary CNS tumors (as noted above), brainstem gliomas account for the largest proportion of deaths at approximately 38%. Patients with a diffuse intrinsic pontine glioma have a median survival of less than 12 months. Clinical presentation Diffuse intrinsic pontine glioma often presents initially as a very large mass. Symptoms typically develop relatively acutely (less than one month), although more subtle symptoms may develop over a period of months. The classic described triad of symptoms includes cranial nerve deficits, ataxia, and long tract signs. Patients may develop signs of obstructive hydrocephalus due to compression of the fourth ventricle. Imaging findings Diagnosis is typically made solely on the base of imaging (due to critical location), though occasionally tissue sampling is done to confirm pathology. CT: Often appears as a hypodense and expansile lesion. MRI: Appears as an expansile, T2-hyperintense, T1-iso/hypointense mass with no/mild enhancement and typically without associated restricted diffusion. Enhancement is associated with a worse prognosis. Diffuse versus focal tumor (focal with better prognosis). References Hipp SJ, Steffen-Smith E, Hammoud D, Shih JH, Bent R, Warren KE. Predicting outcome of children with diffuse intrinsic pontine gliomas using multiparametric imaging. Neuro Oncol. 2011;13(8):904-909. Tisnado J, Young R, Peck KK, Hague S. Conventional and advanced imaging of diffuse intrinsic pontine glioma. J Child Neurol. 2016;31(12):1386-1393.