475 lines
49 KiB
Markdown
475 lines
49 KiB
Markdown
---
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title: "Brain Tumor in Newborn/Infant"
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docid: "12b32579-c99b-41c0-95fd-f2ad1fc4a4fd"
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authors:
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- key: "f184750a-90b4-47a7-907b-23b05d70357a"
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value: "Chang Yueh Ho, MD"
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breadcrumbs:
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-
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name: "Brain"
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slug: "brain"
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treeNodeId: "6d8829f1-14d7-45af-8675-255189aa526a"
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-
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name: "Differential Diagnosis"
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slug: "differential-diagnosis"
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treeNodeId: "a7fdd139-664e-4bb8-8d18-400e4733ff60"
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-
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name: "Brain Parenchyma, General"
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slug: "brain-parenchyma-general"
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treeNodeId: "e79be97b-28c0-4023-be87-334c0579d35d"
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-
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name: "Clinically Based Differentials"
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slug: "clinically-based-differentials"
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treeNodeId: "108519f7-93d7-4662-85dd-2239f2422821"
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-
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name: "Brain Tumor in Newborn/Infant"
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slug: "brain-tumor-in-newborninfant"
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treeNodeId: null
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category: "Brain"
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documentVersionId: "6d309907-eb33-42ec-abac-50970b8269ba"
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imageCount: 71
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lastUpdated: "01/25/23"
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pageDescription: "Brain Tumor in Newborn/Infant"
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pageKeywords: "Brain, Differential Diagnosis, Brain Parenchyma, General, Clinically Based Differentials, Brain Tumor in Newborn/Infant"
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pageTitle: "Brain Tumor in Newborn/Infant | STATdx"
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enhancedTitle: "Brain Tumor in Newborn/Infant"
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type: "DDX"
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references: true
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breadcrumbs:
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- "Brain"
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- "Differential Diagnosis"
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- "Brain Parenchyma, General"
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- "Clinically Based Differentials"
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- "Brain Tumor in Newborn/Infant"
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---
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# ESSENTIAL INFORMATION
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- ## Key Differential Diagnosis Issues
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- Newborn/infant brain tumors
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- Typically large, bulky, inhomogeneous
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- 60-70% supratentorial
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- Infratentorial more common in older children
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- Immature, high-grade tumors more common
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- ## Helpful Clues for Common Diagnoses
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- **Teratoma**
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- Most common fetal and congenital brain tumor
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- Midline, supratentorial
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- Small, lobular or holocranial
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- Contents
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- Ca⁺⁺, cysts
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- Fat in mature teratoma, less commonly in immature teratoma; enhancing soft tissue
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- Look for associated congenital brain anomalies
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- **Infant-Type Hemispheric Glioma**
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- Large, heterogeneous, hemispheric
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- High-grade cellular astrocytoma
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- Better outcome than other histone-associated pediatric high-grade gliomas
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- **Medulloblastoma**
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- SHH-activated and non-WNT/non-SHH (group 3) more common in infants
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- Posterior fossa mass with hydrocephalus
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- Restricts on DWI (best MR clue)
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- Enhancement usual (may be late/slow)
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- Sparse Ca⁺⁺: ~ 20%; hemorrhage rare
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- Hypercellularity reflected on imaging
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- Hyperdense (NECT), hypointense (T2)
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- SHH-activated
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- Cerebellar hemisphere, not centered in 4th ventricle
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- Intense enhancement
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- Additional *TP53* mutation carries poor prognosis
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- Group 3
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- Classic 4th ventricular location
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- Enhancement common, group 4 has less enhancement
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- Poor prognosis when presenting with dissemination
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- **Ependymoma,****Posterior Fossa Type A**
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- Posterior fossa A ependymomas characterized by ↓ H3 K27 expression
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- Younger children, poor outcome
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- Lateral in 4th ventricle, extends through foramina of Luschka
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- Heterogeneous enhancement
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- Ca⁺⁺ ± hemorrhage
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- **Supratentorial Ependymoma**
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- Periventricular/extraventricular > intraventricular
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- Derived from periventricular ependymal rests
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- Large, bulky; Ca⁺⁺: ~ 50%
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- Variable necrosis, hemorrhage
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- *ZFTA* fusion-positive: Seen in infants and older children, poor prognosis
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- *YAP1* fusion-positive: Usually seen in infants, good prognosis
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- **Choroid Plexus Papilloma**
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- Choroid plexus papilloma (CPP): Lobulated intraventricular mass
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- Lateral > 4th > 3rd
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- NECT: Isointense to dense
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- Isointense to slightly hyperintense on T2WI
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- Vividly enhancing
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- Hydrocephalus common
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- ## Helpful Clues for Less Common Diagnoses
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- **Pilocytic Astrocytoma, Pilomyxoid Variant**
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- Younger age presentation than typical pilocytic astrocytoma (PA)
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- Often presents as large, enhancing, infiltrative mass involving optic pathway
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- Despite low-grade tumor, it can have leptomeningeal seeding
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- **Atypical Teratoid-Rhabdoid Tumor**
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- Medulloblastoma-like, +
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- Metastases at diagnosis more common
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- Cysts, hemorrhage more common
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- Variable contrast enhancement
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- Cerebellopontine angle cistern location more common
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- Seeding via CSF pathway common
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- **CNS Embryonal Tumor**
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- Previously primitive neuroectodermal tumor (PNET)
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- Large, complex mass
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- Restricts on DWI
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- Heterogeneous signal enhancement
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- Ca⁺⁺ more common than in posterior fossa PNETs
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- Hemorrhage, necrosis common
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- Hemispheric
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- Mean diameter: 5 cm
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- Especially newborn/infants
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- Minimal peritumoral edema
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- Suprasellar: Early neuroendocrine, visual disturbances
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- **Desmoplastic Infantile Ganglioglioma/Astrocytoma**
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- Desmoplastic infantile gangliogliomas(DIGs)/astrocytoma often have large cyst
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- Cortically based, enhancing tumor nodule
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- Enhancing adjacent pia and dura; low grade
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- Good outcome with complete surgical resection
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- ## Helpful Clues for Rare Diagnoses
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- **Choroid Plexus Carcinoma**
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- Similar to CPP, +
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- Brain invasion; Ca⁺⁺, cysts, bleed
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- Ependymal, subarachnoid space seeding (can be seen with both CPP, choroid plexus carcinoma)
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- **Embryonal Tumor With Multilayered Rosettes**
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- Rare malignant embryonal brain tumor
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- Young children (< 5 years)
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- Histologic differentiation varies
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- Neuronal, astrocytic, ependymal, melanotic, etc.
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- Imaging appearance reflects variable differentiation
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- Medulloepithelioma, ependymoblastoma, and embryonal tumor with abundant neuropil and true rosettes (ETANTR) all have similar molecular features and are grouped as embryonal tumor with multilayered rosettes (ETMR)
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- **Neurocutaneous Melanosis (Melanoma/Melanocytoma)**
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- Giant or multiple cutaneous melanocytic nevi, +
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- Melanosis: Bright T1 lesions in amygdala, cerebellum without fat saturation
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- T2 hypointense to isointense, no enhancement
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- Melanoma: Melanosis + diffuse leptomeningeal enhancement
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- Degeneration into malignant melanoma common
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## References
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# Selected References
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1. [Louis DN et al: The 2021 WHO Classification of Tumors of the Central Nervous System: a summary. Neuro Oncol. 23(8):1231-51, 2021](http://www.ncbi.nlm.nih.gov/pubmed/?term=34185076%5Bpmid%5D)
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1. [Clarke M et al: Infant high-grade gliomas comprise multiple subgroups characterized by novel targetable gene fusions and favorable outcomes. Cancer Discov. 10(7):942-63, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32238360%5Bpmid%5D)
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1. [Lambo S et al: ETMR: a tumor entity in its infancy. Acta Neuropathol. 140(3):249-66, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32601913%5Bpmid%5D)
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1. [Gessi M et al: Medulloblastoma with extensive nodularity: a tumor exclusively of infancy? Neuropathol Appl Neurobiol. 43(3):267-70, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=26990710%5Bpmid%5D)
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1. [Shekdar KV et al: Brain tumors in the neonate. Neuroimaging Clin N Am. 27(1):69-83, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=27889024%5Bpmid%5D)
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1. [Munjal S et al: Infant brain tumours: a tale of two cities. Childs Nerv Syst. 32(9):1633-40, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=27299432%5Bpmid%5D)
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1. [Kralik SF et al: Diffusion imaging for tumor grading of supratentorial brain tumors in the first year of life. AJNR Am J Neuroradiol. 35(4):815-23, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24200900%5Bpmid%5D)
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## Images
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### Selected Images
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**Teratoma**
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*Axial T1 C+ MR shows a large, enhancing mass in a temporal off-midline location <img src='img/arrows/CS.png'/> in a neonate with maximal hydrocephalus and layering blood products <img src='img/arrows/CO.png'/> as well as cellular debris <img src='img/arrows/CC.png'/>.*
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**Teratoma**
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*Axial T1 C+ MR shows a large, enhancing mass in a temporal off-midline location <img src='img/arrows/CS.png'/> in a neonate with maximal hydrocephalus and layering blood products <img src='img/arrows/CO.png'/> as well as cellular debris <img src='img/arrows/CC.png'/>.*
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**Teratoma**
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*Axial T1 C+ MR shows a large, enhancing mass in a temporal off-midline location <img src='img/arrows/CS.png'/> in a neonate with maximal hydrocephalus and layering blood products <img src='img/arrows/CO.png'/> as well as cellular debris <img src='img/arrows/CC.png'/>.*
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**Teratoma**
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*Axial T1 C+ MR shows a large, enhancing mass in a temporal off-midline location <img src='img/arrows/CS.png'/> in a neonate with maximal hydrocephalus and layering blood products <img src='img/arrows/CO.png'/> as well as cellular debris <img src='img/arrows/CC.png'/>.*
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**Teratoma**
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*Coronal T2 MR shows the heterogeneous mass in the left middle cranial fossa <img src='img/arrows/CS.png'/> with severe hydrocephalus and a thinned brain mantle <img src='img/arrows/CO.png'/>. Fetal teratomas often have poor outcome due to compression of the brain.*
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**Infant-Type Hemispheric Glioma**
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*Axial T2 MR shows a large, heterogeneous mass with central necrosis and blood products <img src='img/arrows/CS.png'/> causing midline shift <img src='img/arrows/CO.png'/> and hydrocephalus <img src='img/arrows/CC.png'/>.*
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**Infant-Type Hemispheric Glioma**
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*Axial T1 C+ MR shows heterogeneous enhancement of the large left frontal lobe. In an infant, this is consistent with an infant-type hemispheric glioma, a high-grade tumor characterized by NTRK, ROS1, ALK, or MET alteration of tyrosine kinases.*
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**Medulloblastoma**
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*Axial T1 C+ MR shows multiple nodules in the right middle cerebellar peduncle <img src='img/arrows/CS.png'/> and vermis <img src='img/arrows/CO.png'/> in an infant. This was an SHH desmoplastic medulloblastoma. Desmoplastic histology is associated with SHH pathway alteration and can be nodular in appearance.*
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**Medulloblastoma**
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*Axial b=1000 DWI MR shows the nodular masses <img src='img/arrows/CS.png'/> have decreased diffusion compatible with high-grade neoplasm. SHH medulloblastomas have variable outcomes with a TP53 mutation associated with poor outcome.*
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**Ependymoma, Posterior Fossa Type A**
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*Axial T2 MR shows a heterogeneous mass in the 4th ventricle extending laterally through the left foramen of Luschka <img src='img/arrows/CS.png'/>. This was a posterior fossa type A (PFA) ependymoma at resection. Ependymomas have a "toothpaste" propensity to extend through the 4th ventricular foramina.*
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**Ependymoma, Posterior Fossa Type A**
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*Axial T1 C+ MR shows the heterogeneous enhancement of the PFA ependymoma. PFA ependymomas tend to occur in young children and carry a worse prognosis than posterior fossa type B (PFB) ependymomas.*
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**Supratentorial Ependymoma**
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*Axial T2 TSE FS MR shows a heterogeneous mass in a periventricular location <img src='img/arrows/CS.png'/>. Supratentorial ependymomas are thought to arise from periventricular ependymal rests.*
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**Supratentorial Ependymoma**
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*Axial T1 C+ MR shows cystic and heterogeneous enhancement in this ZFTA fusion supratentorial ependymoma. This subtype has a worse prognosis than the more rare YAP1 subgroup, which tends to present in younger children.*
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**Choroid Plexus Papilloma**
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*Axial T2 MR shows a large intraventricular mass coinciding with the left lateral choroid plexus glomus <img src='img/arrows/CS.png'/>. The mass has decreased T2 signal, which may represent Ca⁺⁺ or blood products. There is obstructive hydrocephalus, which is common in choroid plexus tumors.*
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**Choroid Plexus Papilloma**
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*Coronal T1 C+ MR shows a heterogeneous, lobular, enhancing mass in the left lateral ventricle causing obstructive hydrocephalus. At resection, there was an atypical choroid plexus papilloma (CPP), WHO grade 2.*
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**Pilocytic Astrocytoma, Pilomyxoid Variant**
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*Coronal T1 C+ MR shows a large left hemispheric mass with solid enhancement centrally <img src='img/arrows/CS.png'/> and cystic component <img src='img/arrows/CO.png'/> peripherally. Pilomyxoid variant of pilocytic astrocytomas may present in younger patients, involve the optic pathway, and are more locally aggressive.*
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**Pilocytic Astrocytoma, Pilomyxoid Variant**
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*Axial T2 MR shows the mass centered in the central gray nuclei with midline shift <img src='img/arrows/CS.png'/>. The solid component has relative increased T2 content <img src='img/arrows/CO.png'/>, suggesting a low-grade neoplasm.*
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**Pilocytic Astrocytoma, Pilomyxoid Variant**
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*Axial b=1000 DWI MR shows increased diffusion of the solid tumor portions <img src='img/arrows/CS.png'/>, consistent with the low-grade nature of pilomyxoid astrocytoma. In infants, DWI is helpful in differentiating high- vs. low-grade tumors that tend to be large, heterogeneous, and hemispheric.*
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**Atypical Teratoid-Rhabdoid Tumor**
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*Sagittal T1 C+ MR shows 3 nodular masses in the posterior fossa within the cisterna magna <img src='img/arrows/CS.png'/>, 4th ventricle <img src='img/arrows/CO.png'/>, and premedullary cistern <img src='img/arrows/CC.png'/>.*
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**Atypical Teratoid-Rhabdoid Tumor**
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*Axial b=1000 DWI shows the multiple posterior fossa masses <img src='img/arrows/CS.png'/> have decreased diffusion compatible with high-grade neoplasm. Consider atypical teratoid-rhabdoid tumor in infants.*
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**CNS Embryonal Tumor**
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*Axial b=1000 DWI shows the peripheral solid component has decreased diffusion consistent with a hypercellular high-grade tumor <img src='img/arrows/CS.png'/>. CNS embryonal tumors were previously termed primitive neuroectodermal tumors (PNETs).*
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**CNS Embryonal Tumor**
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*Axial T1 C+ MR shows a large left hemispheric mass with a large central proteinaceous cyst with intrinsic T1 shortening <img src='img/arrows/CS.png'/>. The solid peripheral component has minimal enhancement <img src='img/arrows/CO.png'/>.*
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**Desmoplastic Infantile Ganglioglioma/Astrocytoma**
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*Axial T1 C+ MR shows a heterogeneous mass with solid enhancement peripherally with a broad dural base <img src='img/arrows/CS.png'/> and cysts <img src='img/arrows/CO.png'/> medially in an infant. Desmoplastic infantile tumors have a broad dural base as a characteristic appearance.*
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**Desmoplastic Infantile Ganglioglioma/Astrocytoma**
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*Axial T2 TSE MR shows the solid component has a hypointense appearance <img src='img/arrows/CS.png'/>.*
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**Desmoplastic Infantile Ganglioglioma/Astrocytoma**
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*Axial b=1000 DWI MR shows the solid components do not have decreased diffusion <img src='img/arrows/CS.png'/>, correlating with the low-grade nature of this tumor. Infants that have a complete resection have a good outcome.*
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**Choroid Plexus Carcinoma**
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*Axial T1 C+ MR shows a large, heterogeneous mass centered in the left atria of the lateral ventricle <img src='img/arrows/CS.png'/>, consistent with a choroid plexus tumor. This was a carcinoma at resection.*
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**Choroid Plexus Carcinoma**
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*Axial T2 TSE MR shows the large, isointense lateral ventricular mass. Radiographic size, invasion, and dissemination are not helpful to distinguish between a low-grade papilloma and a high-grade carcinoma. Carcinomas have a propensity to present in younger children.*
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**Embryonal Tumor With Multilayered Rosettes**
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*Axial T2 MR demonstrates a homogeneous cortical mass in a young child presenting with seizures. A dysembryoplastic neuroepithelial tumor (DNET) was the favored differential, but this was an embryonal tumor with multilayered rosettes (ETMR) at resection.*
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**Embryonal Tumor With Multilayered Rosettes**
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*Axial ADC map shows an area of decreased diffusion <img src='img/arrows/CS.png'/> within the cortically based tumor, consistent with a high-grade neoplasm. ETMR is typically an aggressive and large high-grade tumor, with this tumor having an atypical early presentation.*
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**Neurocutaneous Melanosis (Melanoma/Melanocytoma)**
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*Coronal T1 MR in this neonate with large segmental cutaneous nevus shows an area of intrinsic T1 shortening in the left cerebellum <img src='img/arrows/CS.png'/>, consistent with neurocutaneous melanosis.*
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**Neurocutaneous Melanosis (Melanoma/Melanocytoma)**
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*Coronal T1 C+ MR shows 2 masses in the amygdala with intrinsic T1 shortening <img src='img/arrows/CS.png'/> compared to precontrast T1 (not shown). Amygdala involvement is a common location for neurocutaneous melanosis.*
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### Additional Images
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**Teratoma**
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*Axial T1 MR in this 7-day-old infant shows T1-bright signal from fat <img src='img/arrows/WS.png'/> scattered throughout the lesion.*
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**Teratoma**
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*Axial NECT in the same child at 15 months old shows a complicated pineal region mass consisting of fat <img src='img/arrows/WO.png'/>, solid tissue <img src='img/arrows/WS.png'/>, and calcification <img src='img/arrows/WC.png'/>.*
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**Medulloblastoma**
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*Axial CECT in a 1-year-old infant with macrocrania and vomiting shows a giant enhancing posterior fossa mass <img src='img/arrows/BS.png'/> that fills the posterior fossa. Note peripheral myxoid or cystic tumor components <img src='img/arrows/WC.png'/>.*
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**Medulloblastoma**
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*Axial T2 MR in a 10-month-old shows a giant isointense posterior fossa mass with distinct, thick, nodular morphology <img src='img/arrows/BS.png'/>. PNET-medulloblastoma with extensive nodularity, a subtype of medulloblastoma, has a somewhat better prognosis.*
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**Medulloblastoma**
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*Axial T2 MR in a 4-month-old shows an intermediate- to low-signal mass that splays and encases posterior communicating <img src='img/arrows/WO.png'/> and superior cerebellar <img src='img/arrows/WS.png'/> arteries.*
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**Medulloblastoma**
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*Axial DWI MR shows diffusion restriction in this tumor <img src='img/arrows/WC.png'/> involving mesencephalon, circummesencephalic cistern, and mesial temporal lobe.*
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**Medulloblastoma**
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*Coronal T1 C+ MR in this 10-month-old shows grape-like nodular enhancement <img src='img/arrows/WS.png'/>. Medulloblastoma with extensive nodularity is a PNET-medulloblastoma variant that has somewhat better prognosis.*
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**Supratentorial Ependymoma**
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*Axial T2 MR in a 12-week-old infant shows a mixed heterogeneity left temporal lobe mass.*
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**Supratentorial Ependymoma**
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*Axial T2* GRE MR shows multifocal hemosiderin and calcific foci <img src='img/arrows/WO.png'/>.*
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**Choroid Plexus Papilloma**
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*Coronal T1 C+ MR shows bilateral choroid plexus papillomas. The left <img src='img/arrows/WC.png'/> is bulky and frond-like, while the right <img src='img/arrows/WO.png'/> is stretched by the associated cyst.*
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**Choroid Plexus Papilloma**
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*Axial T2 MR shows a large cyst <img src='img/arrows/BC.png'/>, coloboma <img src='img/arrows/WO.png'/>, and temporal lobe subependymal heterotopia <img src='img/arrows/WS.png'/> in a 4-day-old girl with Aicardi syndrome.*
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**Supratentorial Ependymoma**
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*Sagittal ultrasound shows a bulky subependymal giant cell astrocytoma <img src='img/arrows/WO.png'/> at the foramen of Monro in this newborn with cardiac rhabdomyoma and tuberous sclerosis. There are multiple additional tubers <img src='img/arrows/WC.png'/> on the same image.*
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**Desmoplastic Infantile Ganglioglioma/Astrocytoma**
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*Coronal T1 C+ MR in a 7-month-old infant shows a massive right frontal cystic tumor with a solid enhancing component that involves the medial frontal cortex <img src='img/arrows/WS.png'/> and falx.*
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**Desmoplastic Infantile Ganglioglioma/Astrocytoma**
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*Axial DWI MR shows a lack of diffusion restriction in the medial cortical tumor rind <img src='img/arrows/WO.png'/>, which enhanced following contrast administration in this infant (not shown).*
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**Choroid Plexus Carcinoma**
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*Axial T1 C+ MR in this 9-month-old infant shows a large, bulky, avidly enhancing left intraventricular tumor <img src='img/arrows/BC.png'/> with invasion of the overlying brain <img src='img/arrows/WO.png'/>. There are multiple intraventricular metastases <img src='img/arrows/BO.png'/>.*
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**Choroid Plexus Carcinoma**
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*AP angiography performed as a part of preoperative embolization shows hypervascularity <img src='img/arrows/BS.png'/> and multiple areas of contrast puddling <img src='img/arrows/BC.png'/>.*
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**Atypical Teratoid-Rhabdoid Tumor**
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*Sagittal T2 MR in this 7-month-old infant shows hydrocephalus and a complicated solid and cystic tumor filling the 4th ventricle, supravermian cistern, and extending through the tentorial incisura <img src='img/arrows/WO.png'/>.*
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**Atypical Teratoid-Rhabdoid Tumor**
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*Coronal T1 C+ MR in the same 7-month-old shows a right frontal metastatic deposit <img src='img/arrows/WO.png'/>.*
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**Neurocutaneous Melanosis (Melanoma/Melanocytoma)**
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*Sagittal T1 MR shows increased signal intensity of the hippocampus <img src='img/arrows/WO.png'/> in this 10-month-old with a large cutaneous nevus. Pachymeningeal thickening <img src='img/arrows/WS.png'/> is present prior to contrast administration.*
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**Embryonal Tumor With Multilayered Rosettes**
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*Sagittal T1 MR in a 5-day-old infant shows a massive hemorrhagic tumor replacing and expanding the upper cervical spinal cord, the brainstem, and the cerebellum. The tumor protrudes through the incisura and displaces the straight sinus <img src='img/arrows/WO.png'/>.*
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**Embryonal Tumor With Multilayered Rosettes**
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*Axial T2 MR shows a well-circumscribed, heterogeneous mass asymmetrically expanding the pons and the right middle cerebellar peduncle.*
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**Embryonal Tumor With Multilayered Rosettes**
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*Axial DWI of the well-circumscribed pontine tumor shows intense increased signal from decreased diffusion, suggesting this is a high-grade neoplasm. Medulloepithelioma is a highly primitive and aggressive tumor with a dismal prognosis.*
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**Atypical Teratoid-Rhabdoid Tumor**
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*Axial T1 C+ MR shows heterogeneous enhancement of the cerebellopontine angle tumor. Posterior fossa atypical teratoid-rhabdoid tumors can be difficult to distinguish from medulloblastomas, and both high-grade tumors have decreased diffusion.*
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**Atypical Teratoid-Rhabdoid Tumor**
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*Axial T2 MR shows a heterogeneous mass with cystic change and low T2 signal in the left cerebellopontine angle with invasion into the left internal auditory canal <img src='img/arrows/CS.png'/>.*
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**Choroid Plexus Carcinoma**
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*Axial T1 C+ MR shows heterogeneous enhancement of the large intraventricular mass. While hemorrhage and parenchymal invasion are more likely with choroid plexus carcinoma, no particular imaging sign is able to specifically differentiate a papilloma from a carcinoma.*
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**Choroid Plexus Carcinoma**
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*Axial T2 MR shows a large lobular, heterogeneous mass centered in the left lateral ventricle. There are fluid-fluid levels <img src='img/arrows/CS.png'/> from layering blood products.*
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**Pilocytic Astrocytoma, Pilomyxoid Variant**
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*Axial T2 MR shows a mass expanding the chiasm and optic tracts <img src='img/arrows/CS.png'/> with mass effect on the medial temporal lobes and midbrain. The T2 hyperintensity suggests a low-grade neoplasm.*
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**Pilocytic Astrocytoma, Pilomyxoid Variant**
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*Sagittal T1 C+ MR shows intense enhancement with central hypointensity <img src='img/arrows/CS.png'/> from infiltration of the optic chiasm and hypothalamus. In very young children, pilomyxoid astrocytomas often present very large in the optic pathway.*
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**Desmoplastic Infantile Ganglioglioma/Astrocytoma**
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*Axial T2 MR shows a heterogeneous mass with central cysts and a hypointense peripheral solid component with broad dural attachment <img src='img/arrows/CS.png'/>. There is adjacent left frontal lobe edema and midline shift.*
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**Desmoplastic Infantile Ganglioglioma/Astrocytoma**
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*Coronal T1 C+ MR shows intense enhancement of the peripheral solid component of the left frontal heterogeneous mass. This peripheral, broad dural attachment of a solid, enhancing nodule <img src='img/arrows/CS.png'/> is a specific sign of a desmoplastic infantile ganglioglioma or astrocytoma in the appropriate age range.*
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**Choroid Plexus Papilloma**
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*Axial CT shows an isodense, lobular, frond-like mass <img src='img/arrows/CS.png'/> centered in the right atria of the lateral ventricle. There are layering blood products <img src='img/arrows/CO.png'/> within enlarged lateral ventricles, indicating hydrocephalus.*
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**Choroid Plexus Papilloma**
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*Coronal T1 C+ MR shows an intensely enhancing lobular mass <img src='img/arrows/CS.png'/> centered in the right choroid plexus glomus consistent with a choroid plexus tumor. There is associated hydrocephalus.*
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**Supratentorial Ependymoma**
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*Axial T1 C+ MR shows heterogeneous peripheral enhancement <img src='img/arrows/CS.png'/> of mass surrounding central cystic necrosis. Supratentorial ependymomas arise most commonly in a periventricular location from ependymal rest cells.*
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**Supratentorial Ependymoma**
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*Axial T2 MR shows a heterogeneous mass with central necrosis in a periventricular location in this infant's frontal lobe. There is a peripheral hypointense T2 component indicating blood products <img src='img/arrows/CS.png'/>. There is ventricular enlargement from hydrocephalus.*
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**Medulloblastoma**
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*Axial T2 MR shows a nodular and invasive mass within the superior 4th ventricle <img src='img/arrows/CS.png'/> and cerebellar vermis <img src='img/arrows/CO.png'/>. There is severe hydrocephalus and transependymal interstitial edema. Note susceptibility artifact from a ventriculostomy reservoir <img src='img/arrows/CC.png'/>.*
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**Medulloblastoma**
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*Sagittal T1 C+ MR shows nodular, grape-like enhancement of the posterior fossa mass filling the superior 4th ventricle <img src='img/arrows/CS.png'/> and invading the vermis <img src='img/arrows/CO.png'/>. This appearance is suggestive of medulloblastoma with extensive nodularity, which has a favorable prognosis.*
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**CNS Embryonal Tumor**
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*Axial ADC map shows decreased diffusion <img src='img/arrows/CS.png'/> surrounding the central necrosis. This was a WHO grade 4 CNS embryonal tumor, not otherwise specified. This was previously called PNET. The 2016 WHO CNS tumor classification has removed the term PNET.*
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**CNS Embryonal Tumor**
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*Axial T1 C+ MR shows a heterogeneous mass in the right cerebral hemisphere, which causes midline shift and ipsilateral ventricular effacement. There is central necrosis with surrounding ring enhancement <img src='img/arrows/CS.png'/>.*
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**Medulloblastoma**
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*Axial DWI MR shows increased signal from decreased diffusion of the 4th ventricular tumor, consistent with a high-grade, hypercellular tumor, such as medulloblastoma. Decreased DWI can be seen in many high-grade neoplasms.*
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**Medulloblastoma**
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*Axial T2 MR shows a heterogeneous tumor within the 4th ventricle consistent with a classic medulloblastoma. Note the CSF cleft <img src='img/arrows/CS.png'/> with the dorsal pons (floor) suggesting attachment to the roof of the 4th ventricle (vermis) <img src='img/arrows/CO.png'/>.*
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**Teratoma**
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*Axial CT shows a midline lobular mass with solid and cystic components with focal coarse calcifications <img src='img/arrows/CS.png'/>. There is extreme hydrocephalus, leaving only a small rind of brain parenchyma <img src='img/arrows/CO.png'/>.*
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**Teratoma**
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*Sagittal T1 MR shows a heterogeneous, lobular midline mass with an area of T1 hyperintensity consistent with areas of fat <img src='img/arrows/CS.png'/>. There is extreme hydrocephalus with macrocrania. Although mature teratomas are low grade, they present very large with poor prognosis due to massive hydrocephalus.*
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**Neurocutaneous Melanosis (Melanoma/Melanocytoma)**
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*Coronal T1 C+ MR in a child with a large congenital nevi shows diffuse patchy and leptomeningeal enhancement from metastatic melanoma. The leptomeningeal form of neurocutaneous melanosis portends a poor prognosis.*
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