Final Pr. ID: Poster #: EDU-084
The 2016 World Health Organization Classification of Tumors of the Central Nervous System has now incorporated molecular and genetic parameters in addition to histology to define many tumor entities. Significant restructuring has occurred for pediatric CNS tumors. For example, medulloblastomas are classified into four genetic subtypes. Other embryonal tumors such as embryonal tumor with multilayered rosettes (ETMR) and atypical teratoid/rhabdoid tumor (ATRT) are further defined by their molecular features. Also new entities have been added defined by both histology and molecular signatures including H3 K27M-mutant diffuse midline glioma, RELA fusion-positive ependymoma and diffuse leptomeningeal glioneuronal tumor (DLGNT).
These more homogeneous and narrowly defined entities are expected to facilitate better classification, prognostication and patient stratification for precision therapy. This also improves the design of clinical trials and experimental models.
In this presentation, we will review the new WHO classification scheme and review the imaging and as well as molecular/genetic features of pediatric CNS tumors.
Radiologists must keep up to date with updates to the WHO classification scheme to be able to better communicate with clinicians ensure optimal patient care and relevant research collaboration.
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Authors: Sato T Shawn , Sato Yutaka
Keywords: WHO, Brain tumors
Henault Kathryn, Kanev Paul, O'loughlin Michael
Final Pr. ID: Poster #: CR-054
Congenital glioblastoma multiforme (cGBM) is the rarest type of congenital brain tumors, constituting approximately 3.5% of cases according to the latest literature, with roughly 60 cases published worldwide. This specific presentation can be defined as ‘definitely congenital’, based on the 1964 classification of congenital tumors by Solitare and Krigman. The case report discusses the clinical presentation, radiologic and histologic findings, treatment, and prognosis of cGBM.
The tumor was first detected on an ultrasound at 31 weeks gestation, performed for a clinical diagnosis of mild oligohydramnios, revealing an unexpected large intracranial lesion. Prior to this finding, the pregnancy course was uneventful - the fetus was naturally conceived by a 30-year-old G2P1001 without history of infection, radiation, or trauma. No concerning maternal past medical history or use of drugs/alcohol during pregnancy. No family history of CNS malignancy was documented. MRI performed at 31 3/7 weeks confirmed a large complex cystic and solid mass lesion, bigger than previously measured on the aforementioned ultrasound, with mixed T1 and T2 signal within the left parietal/occipital lobe. Findings were concerning for a mass lesion, specifically a GBM given the size and complex signal characteristics. A second ultrasound preformed 12 days later showed even further growth, raising suspicion for a rapidly enlarging tumor. A pediatric neurosurgeon and maternal fetal medicine team following the patient had preemptively decided to deliver the fetus at 36 weeks due to increasing macrocephaly, with a plan for postnatal biopsy to establish pathology before a definitive treatment plan was ascertained. Unfortunately the mother presented to the clinic at 33 1/7 weeks with no fetal movement and an ultrasound confirmed intrauterine demise. After a classic Cesarean section, an autopsy confirmed a diagnosis of cGBM. No other congenital abnormalities were revealed.
Although fetal brain tumors are exceedingly rare, it is imperative to diagnose them in-utero due to potential prenatal and postnatal complications. Co-morbidities such as polyhydramnios, spontaneous intracranial hemorrhage, dystocia during delivery, and immediate postnatal heart failure should be continually evaluated for. Knowledge of an intracranial mass will allow providers to appropriately plan the mood of delivery and immediate postnatal course with necessary specialists available for immediate intervention.
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Authors: Henault Kathryn , Kanev Paul , O'loughlin Michael
Keywords: congential brain tumor, glioblastoma multiforme
Final Pr. ID: Poster #: EDU-049
Our purpose is to show representative cases of CNS (Central Nervous System) embryonal tumors in children with discussion of the new WHO (World Health Organization) classification system recently issued in 2016. Read More
Authors: Yoon Hye-kyung , Yoon Hee-mang
Keywords: Pediatric, Brain tumor, Medulloblastoma
Aldraihem Ahmed, Abdeen Nishard
Final Pr. ID: Poster #: SCI-047
To determine the inter-observer agreement in measurement of relative cerebral blood volume (rCBV) in pediatric brain tumors, using normal grey matter as a control. Read More
Authors: Aldraihem Ahmed , Abdeen Nishard
Keywords: brain tumor, T2* perfusion, interobserver agreement
Harreld Julie, Ayaz Muhammad, Hillenbrand Claudia, Loeffler Ralf, Patay Zoltan
Final Pr. ID: Poster #: EDU-072
Detection of leptomeningeal metastasis (LM) is critical to staging and prognosis of childhood CNS cancers like medulloblastoma and ependymoma. 1 Though CSF examination is the historical gold standard for diagnosis, technological advances have earned MRI a central role in metastasis detection; recent work finds MRI more predictive of survival than CSF analysis. 2-4 However, not all sequences are created equal for detection of tumor in the CSF, and pediatric MR imaging presents additional unique challenges such as patient motion, acoustic noise and scan time reduction. In this exhibit, we discuss the strengths and weakness of common sequences for LM detection; technical alternatives for reduction of motion, acoustic noise and scan time; and present a suggested targeted imaging protocol based on current best imaging practice.
With visual examples, we will discuss:
1. Signal characteristics of LM and role of complementary sequences for detection
2. Optimizing scan planes and slice thickness for tumor/metastasis location and patient size
3. Strengths and weaknesses of T1 FLAIR, SE, FSE/TSE, gradient echo and ultrafast spoiled gradient echo (VIBE/FAME/LAVA/THRIVE) sequences in terms of time, resolution, SNR and CSF artifact
4. Utility and optimization of post-contrast FLAIR, DWI, TrueFISP/bSSFP, and subtraction images for metastasis detection
5. Reducing imaging time: targeted sequences, k-space undersampling (HASTE, partial Fourier imaging), parallel imaging
6. Reducing acoustic noise: lowering bandwidth, longer echo spacing, modified gradient wave forms, alternate encoding (PETRA, SWIFT, zero-TE)
7. Reducing motion artifact: 2D vs. 3D, non-Cartesian acquisition schemes, motion correction
References:
1. Engelhard HH, Corsten LA. Cancer Treat Res 2005;125:71-85
2. Maroldi R, Ambrosi C, Farina D. Eur Radiol 2005;15:617-626
3. Pang J, Banerjee A, Tihan T. Journal of neuro-oncology 2008;87:97-102
4. Terterov S, Krieger MD, Bowen I, et al. J Neurosurg Pediatr 2010;6:131-136
Figure: Spine imaging at 3 Tesla. (a) Axial post-contrast T1-weighted VIBE images reconstructed at 3mm/0 gap show leptomeningeal metastasis to excellent advantage, without CSF pulsation artifact which obscures the same metastasis on axial (b) T1 TSE (3mm/0.3mm gap) with contrast. Metastasis was confirmed by CSF analysis.
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Authors: Harreld Julie , Ayaz Muhammad , Hillenbrand Claudia , Loeffler Ralf , Patay Zoltan
Keywords: Brain tumor, MRI, metastasis
Farkas Amy, Hooker Jeffrey, Joyner David
Final Pr. ID: Poster #: EDU-089
Epilepsy can be a progressive, debilitating illness, particularly in the pediatric population. More than half of brain tumors are associated with epilepsy, and 30% of these tumors will not respond to pharmacologic therapy. Recognizing lesions that cause seizures is imperative, as providing an accurate diagnosis can identify patients with surgically treatable disease. In the appropriate patient population, epilepsy surgery can be an effective management option that prevents significant morbidity and mortality from epilepsy and dramatically improves quality of life.
This electronic poster will provide an overview of pediatric epileptogenic tumors including gangliogliomas, dysembryoplastic neuroepithelial tumors, pleomorphic xanthoastrocytoma, papillary glioneuronal tumor, pilocytic astrocytoma, and oligodendroglioma. Cases featuring these tumors and their distinguishing characteristics will be reviewed, as both tumor subtype and location contribute to the epileptogenicity of pediatric brain tumors.
The goal of this poster is to provide a framework for the evaluation of pediatric epileptogenic tumors to establish a focused differential diagnosis when these lesions are identified. This is particularly important for trainees and well as those who do not commonly encounter epileptogenic tumors in their practice.
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Authors: Farkas Amy , Hooker Jeffrey , Joyner David
Keywords: Epilepsy, Brain tumor