Pediatric CNS Tumors Disclosures Whitney Finke, MD Neuroradiology Fellow PGY-6 University of Utah Health Sciences Center Salt Lake City, Utah None Acknowledgements Introduction Nicholas A. Koontz, MD Luke Linscott, MD Tumor location guides your differential diagnosis: Posterior Fossa Sella/Suprasella Supratentorial Intraventricular Temporal Lobe Introduction Tumor location guides your differential diagnosis: Posterior Fossa Sella/Suprasella Supratentorial Intraventricular Temporal Lobe Posterior Fossa Tumors
Case 1 Case 1 7 year old female with progressive ataxia Sagittal T2WI Axial DWI Axial T1WI+C Case 1: Differential Diagnosis Case 1: Differential Diagnosis Atypical teratoid/rhabdoid tumor (AT/RT) Axial DWI Cystic mass with enhancing nodule Arise in cerebellar hemisphere CT: 20% Ca++ MRI: cyst does not FLAIR suppress, DWI negative, solid component +C Coronal T1WI+C Case 1: Differential Diagnosis Case 1: Differential Diagnosis : Midline, round 4 th ventricular mass 0-10 years CT: hyperdense, 20% calcify (Ca++), hemorrhage rare MRI: DWI hyperintense, variable +C 33% CSF spread : Midline, round 4 th ventricular mass 0-10 years CT: hyperdense, 20% calcify (Ca++), hemorrhage rare MRI: DWI hyperintense, variable +C 33% CSF spread Sagittal T1WI+C FS
Case 1: Differential Diagnosis Case 1: Differential Diagnosis Midline, 4 th ventricular tumors Soft/plastic CT: 50% Ca++, +/-cysts and hemorrhage MRI: DWI variable, +C variable Much less likely to disseminate Occur in younger children (0-3 years), peaks at 1 year Similar to medulloblastomas Cysts and hemorrhage are common, may Ca++ CSF spread Axial DWI Case 1: 7 year old female with progressive ataxia Case 1: Differential Diagnosis Sagittal T2WI Axial DWI Axial T1WI+C Axial DWI Companion Case 1.1 Companion Case 1.1 13 year old female with lethargy
Companion Case 1.1 Companion Case 1.1 desmoplastic histologic variant Axial ADC Axial T1WI+C Medulloblastomas: WHO Updates Integrated histologic and genetic parameters Histologic: desmoplastic/nodular, extensive nodularity, large cell, anaplastic Genetic: WNTactivated, SHH activated, group 3, group 4 Louis, D.N., Perry, A., Reifenberger, G. et al. Acta Neuropathol (2016) 131: 803. doi:10.1007/s00401-016-1545-1 Case 2 Case 2 13 year old with headaches and vomiting
Case 2 Case 2: Differential Diagnosis Axial DWI Axial T1WI+C Axial T1WI+C Case 2: Differential Diagnosis Case 2: Pilocytic Axial T1WI+C Cystic mass with enhancing mural nodule Arises from the cerebellum and causes mass effect on the 4 th ventricle Most common posterior fossa tumor in 5-19 year olds Axial T1WI+C Case 3 Case 3 3 year old female with headaches and emesis Axial GRE Axial ADC Axial T1WI+C
Case 3: Differential Diagnosis Case 3 : Differential Diagnosis Case 3: Ependymoma Case 4 Plastic, 4 th ventricular tumor 3 rd most common posterior fossa tumor Bimodal: young children and teens/young adults 4 month old with increased head circumference and fussiness Case 4 Case 4 Axial ADC Axial T1WI+C
Case 4: Differential Diagnosis Case 4: Differential Diagnosis Case 4: AT/RT Case 4: AT/RT WHO Updates <2 % of all pediatric CNS tumors Imaging similar to medulloblastomas Usually off midline Cysts and hemorrhage are common <3 years, peak 1 year Must have alterations of INI1 or rarely BRG1 CNS embyronal tumor with rhabdoid features: histologic features without genetic alterations Case 5 Case 5 9 year old male with new onset strabismus Axial DWI Axial T1WI+C
Case 5: Differential Diagnosis Case 5: Differential Diagnosis Diffuse intrinsic pontine glioma (DIPG) Acute Disseminated Encephalomyelitis Osmotic Demyelination Syndrome Neurofibromatosis Type 1 FASI lesions Diffuse intrinsic pontine glioma (DIPG) Acute Disseminated Encephalomyelitis Osmotic Demyelination Syndrome Neurofibromatosis Type 1 FASI lesions Case 5: DIPG Case 5: DIPG WHO Updates 20-30% of pediatric posterior fossa tumors Expansile pontine mass engulfing the basilar flow void Grades II-IV CT: +/- necrosis, focal areas +C MRI: T2/FLAIR hyperintense, DWI negative, +C variable New Name: Diffuse midline glioma 80% H3 K27Mmutation WHO Grade IV Universally poor prognosis Pediatric Posterior Fossa Tumors Sellar and Suprasellar Pediatric Tumors Diffuse midline gliomas (formerly DIPG)
Case 6 Case 6 9 year old female with a bitemporal visual field defect Case 6 Case 6: Differential Diagnosis /Pilomyxoid Axial T1WI Axial T1WI+C Case 6: Differential Diagnosis Case 6: Differential Diagnosis 50% suprasellar tumors Usually adamantinomatous CT: Complex, cystic mass with Ca++ MRI: T1 hyperintense, T2 variable, FLAIR hyperintense, +C nodular or peripheral Sagittal T1WI+C 90% rule: 90% calcify 90% cystic 90% enhance 90% adamantinomatous Sagittal T1WI+C
Case 6: Differential Diagnosis Case 6: Differential Diagnosis Rare in pediatric patients (1.2%) Can be indistinguishable from craniopharyngiomas Nonenhancing Noncalcified Sagittal T1WI Pituitary Macroadenoma Expansile pituitary mass CT: may have cysts, necrosis, hemorrhage MRI: isointense to gray matter with homogeneous +C Rare in children Sagittal T1WI+C Case 6: Differential Diagnosis Case 6: Differential Diagnosis CT: hyperdense, Ca++ and hemorrhage rare MRI: DWI hyperintense, marked +C 5-10% have additional lesion in pineal region CSF spread common Sagittal T1WI+C /Pilomyxoid Chiasmatic/Hypothal amic or optic pathway glioma Less common location than PF Fusiform optic nerve enlargement vs. rounded cystic mass MRI: FLAIR hyperintense, +C variable Axial FLAIR Case 6: 9 year old female with a bitemporal visual field defect Case 6: 9 year old female with a bitemporal visual field defect /Pilomyxoid /Pilomyxoid
Case 7 Case 7 14 year old male with vision loss Case 7 Case 7 /Pilomyxoid Axial ADC Axial T1WI+C Case 7 Case 7: Germinoma /Pilomyxoid Hyperdense on CT and +DWI 10-12 years M>F, except in sella/suprasellar region Incidence varies based on location: 1-3% in western countries, 11% in Japan
Case 8 Case 8 13 year old female with vision loss Sagittal T2WI Axial FLAIR Axial T1WI+C Case 8 Case 8 /Pilomyxoid /Pilomyxoid Sagittal T2WI Sagittal T2WI Case 8: Pilomyxoid Pediatric Sella/Suprasella Tumors Appearance varies with NF vs. sporadic FLAIR hyperintense, +C variable WHO updates: Not automatically Grade II Overlap with pilocytic, can mature to a Grade I Sagittal T2WI /Pilomyxoid s
Conclusion Tumor location guides your differential diagnosis: Posterior Fossa Sella/Suprasella Supratentorial Intraventricular Temporal Lobe Pediatric CNS Tumors Whitney Finke, MD Department of Radiology University of Utah Health Sciences Center Salt Lake City, Utah