175 lines
15 KiB
Markdown
175 lines
15 KiB
Markdown
---
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title: "CPA Mass, Child"
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docid: "76d2535b-050d-4826-a344-877e5bae4230"
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authors:
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- key: "d19354f3-7ff2-495a-ad3f-064122e45602"
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value: "Bernadette L. Koch, MD"
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- key: "e8af6d26-3aad-47c9-9083-5128aab09af2"
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value: "Susan I. Blaser, MD, FRCPC"
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breadcrumbs:
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-
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name: "Head and Neck"
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slug: "head-and-neck"
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treeNodeId: "ed24ed8c-5d57-4629-879b-447b82d2973d"
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-
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name: "Differential Diagnosis"
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slug: "differential-diagnosis"
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treeNodeId: "40d68862-8975-4dde-ac2b-ebc43ab0fb5c"
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name: "CPA-IAC and Posterior Fossa"
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slug: "cpa-iac-and-posterior-fossa"
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treeNodeId: "c590eedb-4a3b-4158-a04f-ad880564c992"
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-
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name: "Anatomically Based Differentials"
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slug: "anatomically-based-differentials"
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treeNodeId: "debfb06c-8656-4f5d-92c1-eaa468185d78"
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-
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name: "CPA Mass, Child"
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slug: "cpa-mass-child"
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treeNodeId: null
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category: "Head and Neck"
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documentVersionId: "388e4ad1-934e-45b0-8fc0-80caff2df890"
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imageCount: 11
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lastUpdated: "03/28/22"
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pageDescription: "CPA Mass, Child"
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pageKeywords: "Head and Neck, Differential Diagnosis, CPA-IAC and Posterior Fossa, Anatomically Based Differentials, CPA Mass, Child"
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pageTitle: "CPA Mass, Child | STATdx"
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enhancedTitle: "CPA Mass, Child"
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type: "DDX"
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references: true
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breadcrumbs:
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- "Head and Neck"
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- "Differential Diagnosis"
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- "CPA-IAC and Posterior Fossa"
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- "Anatomically Based Differentials"
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- "CPA Mass, Child"
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---
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# ESSENTIAL INFORMATION
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- ## Helpful Clues for Common Diagnoses
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- [Arachnoid Cyst in CPA-IAC](/document/cpa-iac-arachnoid-cyst/f1af2d0f-adfd-49c7-a3b9-8a1c66dce2be)
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- Sharply demarcated extraaxial cyst with CSF density/signal intensity
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- Isointense to CSF on all MR sequences, including complete fluid attenuation on FLAIR images
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- Nonenhancing
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- No diffusion restriction
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- ## Helpful Clues for Less Common Diagnoses
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- [Vestibular Schwannoma](/document/vestibular-schwannoma/48772166-59dc-4909-bc75-538de7dd9ddf)
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- Ovoid when within internal auditory canal (IAC)
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- Ice cream on cone shape when CPA-IAC
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- Hypointense relative to hyperintense CSF on T2 or CISS images, ± intramural cysts
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- Enhancing, well-circumscribed tumor
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- Rare in children, unless neurofibromatosis type 2
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- Infratentorial Ependymoma
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- Heterogeneously enhancing 4th ventricular mass with irregular margins
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- Extends through foramen of Luschka → CPA cistern
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- Calcification in up to 50%
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- [Infantile Hemangioma in CPA-IAC](/document/infantile-hemangioma/4c91584c-4788-4d4d-b854-15d22fdff6b7)
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- Smoothly marginated, intensely enhancing mass in CPA-IAC (proliferating phase)
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- Proliferating, involuting, and involuted phases
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- Usually, involutional timing similar to extracranial hemangiomas; therefore, no surgical intervention
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- Glut-1 marker positive
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- ± PHACE syndrome
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- **P**osterior fossa malformations, **h**emangioma, **a**rterial cerebrovascular anomalies, **c**oarctation of aorta and cardiac defects, **e**ye abnormalities
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- [Epidermoid Cyst in CPA-IAC](/document/cpa-iac-epidermoid-cyst/5e83f596-1ca4-41cb-95aa-469147ca5f8f)
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- Insinuating mass with irregular or scalloped margins
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- ± scalloping brainstem margin
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- Iso- to slightly hyperintense to CSF
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- Nonenhancing, with restricted diffusion
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- ## Helpful Clues for Rare Diagnoses
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- Atypical Teratoid/Rhabdoid Tumor
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- CPA tumor with lytic bone destruction in infants
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- Hypointense compared with CSF on T2, with restricted diffusion, enhancing
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- [Meningioma in CPA-IAC](/document/cpa-iac-meningitis/c3269e91-15d4-4421-917a-216f4d08a038)
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- Dural-based globular or en plaque mass
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- Enhancing mass ± dural tail: 23% of CPA meningiomas have extension/dural tail into IAC
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- Permeative-sclerotic or hyperostotic bone
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- [Lipoma in CPA-IAC](/document/lipoma-in-cpa-iac/3dc1a638-a325-4390-844f-97e01961734d)
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- Ovoid or broad-based against lateral pons
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- Fat signal intensity/density on MR and CT
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- May be mistaken for vestibular schwannoma if T1 C+ images are performed without fat saturation
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- [Metastases in CPA-IAC](/document/cpa-iac-metastases/451451c8-7b49-4ce9-bf22-7c02b4652f23)
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- Irregular, invasive mass; single or multiple
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- Primarily involves leptomeninges, flocculus, choroid plexus, or dura
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- Unilateral or bilateral
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- [Choroid Plexus Papilloma in CPA](/document/choroid-plexus-papilloma/18e712f5-8553-487d-a939-044336cbf0ad)
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- Dumbbell-shaped mass in 4th ventricle, extending through foramen of Luschka → CPA cistern
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- Avid contrast enhancement
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## References
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# Selected References
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1. [Vernon V et al: Surgical management of cerebellopontine angle epidermoid cysts: an institutional experience of 10 years. Br J Neurosurg. 1-10, 2021](http://www.ncbi.nlm.nih.gov/pubmed/?term=33410366%5Bpmid%5D)
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1. [Bartindale M et al: Facial schwannoma management outcomes: a systematic review of the literature. Otolaryngol Head Neck Surg. 163(2):293-301, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32228141%5Bpmid%5D)
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1. [D'Arco F et al: The link between inner ear malformations and the rest of the body: what we know so far about genetic, imaging and histology. Neuroradiology. 62(5):539-44, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32125475%5Bpmid%5D)
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1. [Giordano M et al: Surgical management of cerebellopontine angle arachnoid cysts associated with hearing deficit in pediatric patients. J Neurosurg Pediatr. 21(2):119-23, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29171799%5Bpmid%5D)
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1. [Bonneville F et al: Imaging of cerebellopontine angle lesions: an update. Part 2: intra-axial lesions, skull base lesions that may invade the CPA region, and non-enhancing extra-axial lesions. Eur Radiol. 17(11):2908-20, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=17569053%5Bpmid%5D)
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## Images
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### Selected Images
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**Arachnoid Cyst in CPA-IAC**
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*Axial T2WI MR in a 3-year-old child demonstrates a moderate-sized right extraaxial CPA cyst <img src='img/arrows/WS.png'/>, isointense to intraventricular fluid, moderately compressing the right cerebellar hemisphere and midbrain.*
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**Arachnoid Cyst in CPA-IAC**
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*Axial T2WI MR in a 3-year-old child demonstrates a moderate-sized right extraaxial CPA cyst <img src='img/arrows/WS.png'/>, isointense to intraventricular fluid, moderately compressing the right cerebellar hemisphere and midbrain.*
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**Arachnoid Cyst in CPA-IAC**
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*Axial T2WI MR in a 3-year-old child demonstrates a moderate-sized right extraaxial CPA cyst <img src='img/arrows/WS.png'/>, isointense to intraventricular fluid, moderately compressing the right cerebellar hemisphere and midbrain.*
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**Arachnoid Cyst in CPA-IAC**
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*Axial FLAIR MR in the same patient demonstrates complete attenuation of signal intensity within the cyst <img src='img/arrows/WS.png'/>, typical of an arachnoid cyst. There was no diffusion restriction on DW images (not shown), unlike an epidermoid cyst, which typically demonstrates diffusion restriction.*
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**Vestibular Schwannoma**
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*Axial T1 C+ MR in a 12-year-old boy shows bilateral enhancing CPA-IAC masses <img src='img/arrows/WS.png'/> and a well-defined, enhancing mass involving the left 6th cranial nerve <img src='img/arrows/WC.png'/>, consistent with multiple schwannomas in a child with NF2.*
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**Infratentorial Ependymoma**
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*Axial T1 C+ MR in a 2-year-old child demonstrates a large, heterogeneously enhancing posterior fossa mass <img src='img/arrows/WS.png'/> filling the 4th ventricle, extending through the foramen of Luschka and into the right CPA cistern <img src='img/arrows/WO.png'/>. Notice extension across the midline toward the left foramen of Luschka <img src='img/arrows/WC.png'/>.*
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**Infantile Hemangioma in CPA-IAC**
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*Axial T1 C+ MR in a 1-year-old infant shows a massive facial hemangioma <img src='img/arrows/WS.png'/>, a right CPA-IAC hemangioma <img src='img/arrows/WC.png'/>, and ipsilateral cerebellar hemisphere hypoplasia <img src='img/arrows/WO.png'/>. These findings are consistent with PHACE syndrome.*
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**Epidermoid Cyst in CPA-IAC**
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*Axial T1 C+ MR in a teenager with occipital headaches shows an extraaxial hypointense mass <img src='img/arrows/WS.png'/>, similar in signal intensity to intraventricular CSF, compressing the pons. Differential diagnosis would include arachnoid cyst or dermoid/epidermoid cyst.*
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**Epidermoid Cyst in CPA-IAC**
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*Axial FLAIR MR in the same patient as shows heterogeneous, "dirty" signal intensity within the lesion <img src='img/arrows/WS.png'/> adjacent to the pons, hyperintense relative to CSF. Incomplete suppression on FLAIR sequences is typical of epidermoid cysts.*
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**Epidermoid Cyst in CPA-IAC**
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*Axial DWI MR in the same patient shows hyperintense signal intensity <img src='img/arrows/WS.png'/> relative to intraventricular CSF, which on ADC map proved to be hypointense (not shown), consistent with restricted diffusion typical of epidermoid cysts.*
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### Additional Images
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**Arachnoid Cyst in CPA-IAC**
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*Axial FIESTA in a teenager with epilepsy shows an incidental left CPA arachnoid cyst <img src='img/arrows/WS.png'/>, isointense to intraventricular CSF, without mass effect on the cisternal segments of the 7th/8th cranial nerves <img src='img/arrows/WO.png'/> and without extension into the IAC.*
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**Epidermoid Cyst in CPA-IAC**
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*Axial T2WI MR in a teenager with occipital headaches demonstrates an oblong, T2-hyperintense epidermoid cyst <img src='img/arrows/WS.png'/>, mildly compressing the left ventral pons.*
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**Epidermoid Cyst in CPA-IAC**
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*Axial ADC map in the same patient shows hypointense signal on ADC map image consistent with <img src='img/arrows/WS.png'/> diffusion restriction within the lesion, typical of epidermoid cyst.*
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