315 lines
27 KiB
Markdown
315 lines
27 KiB
Markdown
---
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title: "Posterior Fossa Neoplasm, Adult"
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docid: "9d6bcceb-36a2-4f49-a5b2-1dd076541be8"
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authors:
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- key: "26ebc2e8-e4f7-40ee-8f5c-d23fe383e15c"
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value: "Yoshimi Anzai, MD, MPH"
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- key: "5cff4116-3654-4b3a-bb75-5ebe0b8c9850"
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value: "Anne G. Osborn, MD, FACR"
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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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-
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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: "Posterior Fossa Neoplasm, Adult"
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slug: "posterior-fossa-neoplasm-adult"
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treeNodeId: null
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category: "Head and Neck"
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documentVersionId: "8071b0df-9c2d-4d93-8775-a2b6a68bf9c8"
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imageCount: 22
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lastUpdated: "08/06/18"
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pageDescription: "Posterior Fossa Neoplasm, Adult"
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pageKeywords: "Head and Neck, Differential Diagnosis, CPA-IAC and Posterior Fossa, Anatomically Based Differentials, Posterior Fossa Neoplasm, Adult"
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pageTitle: "Posterior Fossa Neoplasm, Adult | STATdx"
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enhancedTitle: "Posterior Fossa Neoplasm, Adult"
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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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- "Posterior Fossa Neoplasm, Adult"
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---
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# ESSENTIAL INFORMATION
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- ## Key Differential Diagnosis Issues
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- With exception of vestibular schwannoma, posterior fossa (PF) neoplasms rare in adults
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- Most important question: Is lesion intra- or extraaxial
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- **Extraaxial PF lesions**
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- Most adult PF neoplasms are extraaxial
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- By far, most common is **vestibular schwannoma**
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- Meningioma > metastasis > other schwannomas > glomus jugulare paraganglioma
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- **Intraaxial: Parenchymal or intraventricular**
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- Adult parenchymal neoplasms all uncommon/rare
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- Overall, most common by far is metastasis
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- Hemangioblastoma most common primary
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- Glioblastoma, astrocytomas, most common adult supratentorial tumors are rare in PF
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- 4th ventricle
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- Subependymoma > ependymoma > choroid plexus papilloma
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- Number 1 site for ependymoma & subependymoma
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- Ependymoma (usually in children) often extends into foramen of Luschka &/or Magendie
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- Choroid plexus papilloma in body/lateral recess, cerebellopontine angle (CPA)
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- ## Helpful Clues for Common Diagnoses
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- **Vestibular Schwannoma**
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- By far most common adult PF neoplasm
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- All others less common or rare
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- 90% of all CPA-IAC masses
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- Imaging findings
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- Looks like "ice cream on cone" (CPA-IAC)
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- Enhances strongly
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- ± intra- or extratumoral cysts
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- Preserved fluid at IAC fundus is predictive of improved hearing preservation after resection
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- ## Helpful Clues for Less Common Diagnoses
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- **Meningioma****in****Cerebellopontine Angle**
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- Imaging findings
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- Mushroom-shaped mass caps IAC
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- Flat base towards dural surface
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- ± hyperostosis, dural tail sign
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- 25% show IAC involvement
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- **Metastases****in****Cerebellopontine Angle**
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- CPA metastases can arise in 4 locations
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- Dura-arachnoid
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- Cranial nerves (VII, VIII most common)
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- Flocculusmimi
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- Choroid plexus (foramen of Luschka)
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- Imaging findings
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- Irregular, invasive margins
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- Solid, enhancing tumor on dura, cranial nerve surfaces, in flocculus or choroid plexus
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- **Metastasi****s****in Brainstem or Cerebellum**
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- 2nd most common adult PF neoplasm
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- Most common adult parenchymal PF tumor
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- Rarely may be only brain metastasis
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- Enhancing solid or cystic mass with adjacent brain edema
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- **Hemangioblastoma**
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- 95% are located in PF
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- Hemispheres > > vermis > brainstem, 4th ventricle
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- < 50% of patients have von Hippel-Lindau syndrome
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- Look for multiple lesions: Spinal mass, visceral cysts
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- Imaging findings
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- 60% nonenhancing cyst with avidly enhancing mural nodule
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- Large drainage vein, ± blood products
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- **Other Schwannomas**
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- **Trigeminal (CNV)****S****chwannoma**
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- Upper CPA mass
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- Look for dumbbell shape (CPA + Meckel cave components)
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- **Facial****N****erve (CNVII) Schwannoma**
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- CPA-IAC mass with labyrinthine tail
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- Look for CNVII labyrinthine segment tumor
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- If labyrinthine tail absent, mimics vestibular schwannoma
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- **Jugular Foramen****Schwannoma**
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- Enhancing mass arising from jugular foramen
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- Smooth remodeling of bony margins
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- Projects cephalad into CPA cistern
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- **Hypoglossal (CNXII) Schwannoma**
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- Enhancing mass
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- Smooth remodeling or widening of hypoglossal canal
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- Look for ipsilateral tongue atrophy
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- **Subependymoma**
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- Middle-aged/elderly adult; asymptomatic most often
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- Imaging: T2 hyperintense lobulated intraventricular mass
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- 4th ventricle (60%) > other locations
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- ± cysts, calcifications, rare intramural hemorrhage
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- **Choroid Plexus Papilloma**
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- 40% occur in 4th ventricle or CPA cistern in adults
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- Imaging findings
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- Cauliflower- or frond-like excrescences
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- Intense, relatively uniform enhancement
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- ## Helpful Clues for Rare Diagnoses
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- **Astrocytomas**
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- **Glioblastoma**
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- Infratentorial glioblastomas (GBM) rare
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- Typically necrotic, ring enhancing
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- **Anaplastic Astrocytoma**
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- Rare in infratentorial area
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- Infiltrative, variable enhancement
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- **Low-****Grade Diffuse Astrocytoma**
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- Young adults
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- Focal or diffuse T2-hyperintense white matter mass, usually nonenhancing
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- Enhancement suggests conversion to higher grade
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- **Pilocytic Astrocytoma**
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- Rare in adults
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- Cystic mass with enhancing nodule typical
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- **G****lomus Jugulare****Paraganglioma**
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- Spreads superolaterally into middle ear much more frequently than superomedially into CPA
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- Imaging findings
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- Intense heterogeneous enhancement
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- Look for salt and pepper flow voids
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- Permeative; destructive, infiltrative
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- **Dysplastic Cerebellar Gangliocytoma (Lhermitte-Duclos)**
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- May be familial or sporadic
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- Molecular studies suggest high frequency of alterations in PTEN-AKT-mTOR pathway
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- Association with Cowden syndrome
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- Autosomal dominant; germline mutation in *PTEN*tumor suppressor gene
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- ↑ risk of breast, thyroid, genitourinary malignancy, & mucocutaneous lesions
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- Imaging findings
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- Widened, irregular cerebellar folia with ↑ T2
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- Layered/laminated or tiger stripes appearance
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- May cause significant mass effect
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- Typically does not enhance (rarely may)
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- **Medulloblastoma (Desmoplastic Variant)**
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- Desmoplastic variant more common in 2nd-3rd decades
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- Imaging findings
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- Off-midline (lateral cerebellar hemisphere) location
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- Enhances; cerebrospinal fluid spread uncommon
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- **Hemangiopericytoma**
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- Highly cellular & vascularized mesenchymal tumor mimics meningioma
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- Imaging findings
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- Lobular, avidly enhancing, extraaxial mass with dural attachment; ± skull erosion
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- Mimics meningioma, but no calcifications or hyperostosis; often heterogeneous
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- Commonly involve falx, tentorium, or dural sinuses; occipital most common
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- Dural tail seen in ~ 50%
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- **Ecchordosis Physaliphora**
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- Small, gelatinous mass considered ectopic notochordal remnant
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- Midline of craniospinal axis from dorsum sellae to sacrococcygeal region
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- Erosion dorsal clivus to PF
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- Found in 2% of autopsies, typically asymptomatic
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- Imaging findings
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- Hypointense on T1, hyperintense on T2
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- No enhancement
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- ± stalk-like connection to mass
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## References
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# Selected References
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1. [Moinuddin FM et al: Bilateral lateral ventricular subependymoma with extensive multiplicity presenting with hemorrhage. Neuroradiol J. 31(1):27-31, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=28696174%5Bpmid%5D)
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1. [Shrot S et al: Dysplasia and overgrowth: magnetic resonance imaging of pediatric brain abnormalities secondary to alterations in the mechanistic target of rapamycin pathway. Neuroradiology. 60(2):137-150, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29279945%5Bpmid%5D)
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1. [Jiang T et al: Lhermitte-Duclos disease (dysplastic gangliocytoma of the cerebellum) and Cowden syndrome: clinical experience from a single institution with long-term follow-up. World Neurosurg. 104:398-406, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28479525%5Bpmid%5D)
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1. [Ciccarino P et al: Multifocal presentation of medulloblastoma in adulthood. J Neurooncol. 107(2):233-7, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=22071791%5Bpmid%5D)
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1. [Solis OE et al: Rosette-forming glioneuronal tumor: a pineal region case with IDH1 and IDH2 mutation analyses and literature review of 43 cases. J Neurooncol. 102(3):477-84, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=20872044%5Bpmid%5D)
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1. [Goddard JC et al: Fundal fluid as a predictor of hearing preservation in the middle cranial fossa approach for vestibular schwannoma. Otol Neurotol. 31(7):1128-34, 2010](http://www.ncbi.nlm.nih.gov/pubmed/?term=20657334%5Bpmid%5D)
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1. [Koyfman SA et al: Stereotactic radiosurgery for single brainstem metastases: the cleveland clinic experience. Int J Radiat Oncol Biol Phys. 78(2):409-14, 2010](http://www.ncbi.nlm.nih.gov/pubmed/?term=20133072%5Bpmid%5D)
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1. [Andres RH et al: Lhermitte-Duclos disease with atypical vascularization--case report and review of the literature. Clin Neuropathol. 28(2):83-90, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19353838%5Bpmid%5D)
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1. [Bonneville F et al: Imaging of cerebellopontine angle lesions: an update. Part 1: enhancing extra-axial lesions. Eur Radiol. 17(10):2472-82, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=17562049%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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1. [Recinos PF et al: Brainstem tumors: where are we today? Pediatr Neurosurg. 43(3):192-201, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=17409788%5Bpmid%5D)
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1. [Koeller KK et al: From the archives of the AFIP: medulloblastoma: a comprehensive review with radiologic-pathologic correlation. Radiographics. 23(6):1613-37, 2003](http://www.ncbi.nlm.nih.gov/pubmed/?term=14615567%5Bpmid%5D)
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1. [Zabek M: Primary posterior fossa tumours in adult patients. Folia Neuropathol. 41(4):231-6, 2003](http://www.ncbi.nlm.nih.gov/pubmed/?term=14977253%5Bpmid%5D)
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## Images
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### Selected Images
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**Vestibular Schwannoma**
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*Axial T1 C+ MR shows a partly cystic and solid, heterogeneously enhancing, vestibular schwannoma <img src='img/arrows/WC.png'/> involving the right cerebellopontine angle cistern and extending into the internal auditory canal <img src='img/arrows/WS.png'/>.*
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**Vestibular Schwannoma**
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*Axial T1 C+ MR shows a partly cystic and solid, heterogeneously enhancing, vestibular schwannoma <img src='img/arrows/WC.png'/> involving the right cerebellopontine angle cistern and extending into the internal auditory canal <img src='img/arrows/WS.png'/>.*
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**Vestibular Schwannoma**
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*Axial T1 C+ MR shows a partly cystic and solid, heterogeneously enhancing, vestibular schwannoma <img src='img/arrows/WC.png'/> involving the right cerebellopontine angle cistern and extending into the internal auditory canal <img src='img/arrows/WS.png'/>.*
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**Meningioma in Cerebellopontine Angle**
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*Axial T1 C+ MR shows a large, mushroom-shaped, enhancing mass <img src='img/arrows/BS.png'/> in the right cerebellopontine angle cistern. The mass has a broad base toward the dural surface. Note the dural tail sign <img src='img/arrows/WO.png'/> of reactive meningeal thickening in the internal auditory canal.*
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**Metastases in Cerebellopontine Angle**
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*Axial T2 MR in a female patient with breast carcinoma shows a lobulated extraaxial mass in the right flocculus <img src='img/arrows/BS.png'/> with associated parenchymal edema <img src='img/arrows/WC.png'/>. Note the normal flocculus on the left <img src='img/arrows/WO.png'/>.*
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**Metastasis in Brainstem or Cerebellum**
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*Coronal T1 C+ MR demonstrates a partly cystic, partly solid enhancing mass in the left cerebellar hemisphere <img src='img/arrows/WS.png'/> with surrounding vasogenic edema. A 2nd enhancing mass in the left frontal lobe <img src='img/arrows/WC.png'/> increases suspicion for metastatic disease in this patient with lung cancer (confirmed histologically).*
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**Hemangioblastoma**
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*Axial T1 C+ MR in a patient with von Hippel-Lindau syndrome shows a cystic cerebellar mass with a nodule of enhancement <img src='img/arrows/WS.png'/>, typical of hemangioblastoma. Retinal angiomas and an additional posterior fossa hemangioblastoma were also present (not shown).*
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**Jugular Foramen Schwannoma**
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*Axial T1 C+ MR shows a large, solid, intensely enhancing, extraaxial mass extending into the enlarged, smoothly remodeled jugular foramen <img src='img/arrows/WS.png'/>. Intratumoral cysts, not present here, are common in posterior fossa schwannomas.*
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**Subependymoma**
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*Axial T2 MR shows an unusually large lateral ventricle subependymoma <img src='img/arrows/WS.png'/> causing obstructive hydrocephalus. Note the heterogeneous internal signal intensity. No enhancement was seen (not shown), typical of subependymomas. Subependymomas are more often small, incidentally discovered masses.*
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**Choroid Plexus Papilloma**
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*Coronal T1 C+ MR in a 43-year-old woman with headaches shows a "speckled" or "bubbly," heterogeneously enhancing mass in the 4th ventricle <img src='img/arrows/WS.png'/> with extension into the lateral recess <img src='img/arrows/WO.png'/>.*
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**Astrocytomas**
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*Axial T1 C+ MR in an older teenager with nausea and vomiting shows an inhomogeneously enhancing vermian mass <img src='img/arrows/WS.png'/> with cystic and solid components. Preoperative diagnosis was malignant astrocytoma. However, a WHO grade II tumor was found at biopsy.*
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**Low-Grade Diffuse Astrocytoma**
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*Sagittal T2 MR in a 25-year-old woman with lower cranial nerve palsies shows a dorsally exophytic pontomedullary mass <img src='img/arrows/WS.png'/>. Biopsy proved WHO grade II astrocytoma.*
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**Dysplastic Cerebellar Gangliocytoma (Lhermitte-Duclos)**
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*Axial T2 MR shows enlargement and hyperintensity involving the left cerebellar hemisphere <img src='img/arrows/WS.png'/>. Note the classic tiger stripes appearance with overall preserved cerebellar foliar architecture.*
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**Medulloblastoma (Desmoplastic Variant)**
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*Axial T2 MR in a 26-year-old man shows an inhomogeneously hyperintense mass in the lateral cerebellum <img src='img/arrows/WS.png'/>. The mass enhanced heterogeneously (not shown). This is typical of desmoplastic medulloblastoma, although an atypical teratoid rhabdoid tumor could be considered in a child.*
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**Hemangiopericytoma**
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*Axial T1 C+ MR shows a large, inhomogeneously enhancing, destructive, transcalvarial mass with both intracranial <img src='img/arrows/BO.png'/> and extracranial <img src='img/arrows/BC.png'/> components.*
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**Ecchordosis Physaliphora**
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*Sagittal T1 MR shows a midline mass <img src='img/arrows/WS.png'/> in front of and indenting the pons <img src='img/arrows/WO.png'/>. Note the slightly increased signal of the mass relative to CSF. There is loss of a normal hypointense cortical margin in the clivus <img src='img/arrows/WC.png'/> from which the mass originates. The mass was extremely hyperintense on T2 MR, consistent with its notochordal remnant origin.*
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### Additional Images
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**Metastases in Cerebellopontine Angle**
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*Axial T1 C+ MR in a patient with adenocarcinoma shows enhancing metastatic lesions in both IAC cisterns <img src='img/arrows/WS.png'/>. Note the transmodiolar extension on left <img src='img/arrows/WC.png'/>.*
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**Metastasis in Brainstem or Cerebellum**
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*Axial T1 C+ FS MR shows a large, enhancing, cerebellar mass <img src='img/arrows/WS.png'/>. A 2nd mass in the left temporal lobe <img src='img/arrows/WC.png'/> makes a diagnosis of metastases in a patient with known renal cell carcinoma easy.*
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**Hemangioblastoma**
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*Axial T1 C+ FS MR shows a mixed, solid, cystic enhancing mass in the cerebellum <img src='img/arrows/BO.png'/>. Note the enlarged vessels <img src='img/arrows/WS.png'/>, suggesting hypervascularity. A solid/mixed solid lesion with intratumoral cysts is less common than classic hemangioblastoma (a nodule and nonenhancing cyst).*
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**Subependymoma**
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*Sagittal T2 MR shows a small, mildly hyperintense mass <img src='img/arrows/WO.png'/> in the inferior 4th ventricle, found incidentally in this 43-year-old man with headache and trigeminal neuralgia. No hydrocephalus was identified. Diagnosis was presumed subependymoma.*
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**Glomus Jugulare Paraganglioma**
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*Axial T1 C+ FS MR shows a large, strongly enhancing, glomus jugulare paraganglioma <img src='img/arrows/WS.png'/> centered in the jugular foramen. The destructive, erosive nature of the tumor is readily apparent <img src='img/arrows/WC.png'/> with extension into the EAC <img src='img/arrows/WO.png'/>.*
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**Dysplastic Cerebellar Gangliocytoma (Lhermitte-Duclos)**
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*Coronal T2 MR shows enlarged, dysplastic-appearing cerebellar folia with a striated, mixed hyper-/isointense mass in the right cerebellum <img src='img/arrows/WS.png'/>.*
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*Axial T1 C+ MR in a 50-year-old man with supratentorial primary CNS lymphoma shows a subependymal/choroid plexus <img src='img/arrows/BO.png'/> as well as dural-based spread <img src='img/arrows/BS.png'/> of the tumor.*
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**Ecchordosis Physaliphora**
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*Axial T2 FS MR shows a lobulated, hyperintense, extraaxial mass <img src='img/arrows/WS.png'/> indenting the pons. Note the subtle clival involvement <img src='img/arrows/WO.png'/> with focal destruction of a bony cortex typical of ecchordosis physaliphora.*
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