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---
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title: "Craniopharyngioma"
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docid: "00e66680-6731-4287-b5a1-3f0b3f09053b"
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breadcrumbs:
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- "Brain"
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- "Diagnosis"
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- "Anatomy-Based Diagnoses"
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- "Sella and Pituitary"
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- "Neoplasms"
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- "Craniopharyngioma"
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---
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# KEY FACTS
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- ## Terminology
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- Benign, partially cystic sellar region tumor derived from remnants of craniopharyngeal duct/Rathke pouch epithelium
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- 2 types
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- Adamantinomatous (cystic mass in childhood)
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- Papillary (solid mass in older adults)
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- ## Imaging
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- General features
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- Multilobulated, often large (> 5 cm)
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- Occasionally giant, multicompartmental
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- CT: Cystic (90%), Ca⁺⁺ (90%), enhancing (90%)
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- MR: Signal varies with cyst contents
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- Cysts variably hyperintense on T1WI and T2WI
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- Solid portions enhance heterogeneously; cyst walls enhance strongly
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- Cyst contents show broad lipid peak (0.9-1.5 ppm) on MR spectroscopy
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- ## Pathology
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- Most common pediatric intracranial tumor of nonglial origin
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- WHO grade 1
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- ## Clinical Issues
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- Bimodal age distribution
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- Peak 5-15 years; adults 45-60 years (commonly papillary)
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- Pediatric patient with morning headache, visual defect, short stature
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- Endocrine disturbances include growth hormone (GH) deficiency, luteinizing hormone (LH)/follicle-stimulating hormone (FSH) deficiency
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- Others = hypothyroidism > adrenal failure > diabetes insipidus
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- Surgical resection is primary therapy
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- Surgery, radiation therapy, or cyst aspiration for recurrent tumors
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# TERMINOLOGY
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- ## Abbreviations
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- Craniopharyngioma (CP)
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- ## Synonyms
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- Craniopharyngeal duct tumor, Rathke pouch tumor, adamantinoma
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- ## Definitions
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- Benign, partially cystic sellar region tumor derived from Rathke pouch epithelium
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- 2 histologies: Adamantinomatous and papillary
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# IMAGING
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- ## General Features
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- ### Best diagnostic clue
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- CT: Partially Ca⁺⁺ mixed solid/cystic suprasellar mass in child
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- MR: Complex signal intensity suprasellar mass
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- ### Location
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- Surgical division of CPs into 3 groups
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- Sellar
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- Prechiasmatic
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- Retrochiasmatic
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- Imaging locations of CPs (adamantinomatous type)
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- Suprasellar (75%)
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- Suprasellar + intrasellar component (21%)
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- Entirely intrasellar (4%)
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- Often extends into multiple cranial fossae: Anterior (30%), middle (23%), posterior, &/or retroclival (20%)
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- Rare ectopic locations
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- Optic chiasm, 3rd ventricle
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- Other: Nasopharynx, paranasal sinuses, pineal gland, sphenoid (clivus), cerebellopontine angle
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- ### Size
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- Variable; often large at presentation (> 5 cm)
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- Occasionally giant, multicompartmental
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- ### Morphology
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- Multilobulated, multicystic
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- ## CT Findings
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- ### NECT
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- Adamantinomatous type (90% rule)
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- 90% mixed solid (isodense), cystic (hypodense)
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- 90% calcify
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- 90% enhance (solid = nodule; rim = capsule)
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- Papillary type: Often solid, isodense, rarely calcifies
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- ## MR Findings
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- ### T1WI
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- Signal varies with cyst contents
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- Short T1 due to high protein content
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- Classic (adamantinomatous type)
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- Hyperintense cyst + heterogeneous nodule
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- Less common (papillary type)
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- Isointense solid component
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- ### T2WI
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- Cysts are variably hyperintense
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- Solid component = heterogeneous (iso-/hyperintense, Ca⁺⁺ portions hypointense)
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- Hyperintense signal in brain parenchyma adjacent to tumor may indicate
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- Gliosis, tumor invasion, irritation from leaking cyst fluid
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- Edema from compression of optic chiasm/tracts
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- Hypointense T2* = Ca⁺⁺
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- ### FLAIR
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- Cyst contents typically hyperintense
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- ### DWI
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- Variable depending upon character of cyst fluid
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- ### T1WI C+
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- Solid portions enhance heterogeneously; cyst walls enhance strongly
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- ### MRA
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- Vascular displacement &/or encasement
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- ### MRS
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- Cyst contents show broad lipid spectrum (0.9-1.5 ppm)
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- ## Imaging Recommendations
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- ### Best imaging tool
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- MR with thin sagittal, coronal sequences
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- ### Protocol advice
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- Pre-/postcontrast T1WI, T2, FLAIR, GRE, DWI, MRS
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# DIFFERENTIAL DIAGNOSIS
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- [Rathke Cleft Cyst](/document/rathke-cleft-cyst/8f1561f7-92a7-485c-a0ae-2e2d5c8c1628)
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- Noncalcified, less heterogeneous
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- Look for intracystic nodule on T2
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- Does not enhance
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- Claw sign (enhancing pituitary draped around cyst)
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- Small Rathke cleft cyst (RCC) may be indistinguishable from rare intrasellar CP
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- RCCs express CK8 and CK20 (CPs generally do not)
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- ## Suprasellar Arachnoid Cyst
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- No Ca⁺⁺, enhancement
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- ## Hypothalamic/Chiasmatic Astrocytoma
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- Solid or with small cystic/necrotic components
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- Ca⁺⁺ is rare; robust enhancement is common
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- ## Pituitary Adenoma
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- Rare in prepubescent children
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- Isointense with brain
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- Enhances strongly
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- Can mimic CP when cystic and hemorrhagic
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- ## Epidermoid/Dermoid Tumors
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- Minimal or no enhancement
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- ## Thrombosed Aneurysm
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- Contains blood products; use SWI
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- Look for residual patent lumen, phase artifact
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- [Germinoma or Mixed Germ Cell Tumor With Cystic Component(s)](/document/germinoma/078b68a2-67de-457e-818a-63655cec95aa)
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- Cerebrospinal fluid spread is common, Ca⁺⁺ is rare
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# PATHOLOGY
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- ## General Features
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- ### Etiology
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- 2 proposed theories
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- CPs arise from remnants of craniopharyngeal duct and Rathke pouch epithelium
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- CPs arise from squamous epithelial cells in pars tuberalis of adenohypophysis
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- ### Genetics
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- No known genetic susceptibility (rare reports of siblings, parent-child)
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- Small subset of CPs are monoclonal tumors that arise from oncogenes at specific loci
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- Adamantinomatous: *CTNNB1* mutations and aberrant nuclear expression of β-catenin in up to 95% of cases
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- Papillary:*BRAF* V600E mutations in 81-95% of cases
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- ## Staging, Grading, & Classification
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- WHO grade 1
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- MIB-1 labeling index > 7% predicts recurrence
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- ## Gross Pathologic & Surgical Features
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- Solid tumor with variable cysts
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- Adamantinomatous cysts often contain thick "crankcase oil" fluid
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- Epithelial fronds penetrate adjacent hypothalamus/chiasm
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- ## Microscopic Features
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- Adamantinomatous (mostly pediatric)
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- Multistratified squamous epithelium with nuclear palisading
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- Nodules of "wet" keratin
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- Dystrophic Ca⁺⁺
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- Papillary (mostly adults)
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- Sheets of squamous epithelium form pseudopapillae
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- Villous fibrovascular stroma
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- Malignant transformation, distant metastases rare
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- May occur with varied histologies, resulting in poor prognosis
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# CLINICAL ISSUES
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- ## Presentation
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- ### Most common signs/symptoms
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- Symptoms vary with location, size of tumor, age of patient
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- Visual disturbances (60-85%)
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- Bitemporal hemianopsia
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- ### Other signs/symptoms
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- Endocrine disturbances (52-87%)
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- Growth hormone (GH) deficiency (75%) > luteinizing hormone (LH)/follicle-stimulating hormone (FSH) deficiency > hypothyroidism > adrenal failure > diabetes insipidus
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- Headaches
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- Cognitive impairment (~ 50%)
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- ### Clinical profile
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- Pediatric patient with morning headache, visual defect, short stature
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- ## Demographics
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- ### Age
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- Bimodal distribution (peak 5-15 years, with smaller peak 45-60 years)
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- Papillary CP: 40-55 years
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- ### Sex
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- M = F
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- ### Ethnicity
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- More common in Japanese children
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- ### Epidemiology
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- Most common pediatric intracranial tumor of nonglial origin
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- Comprise 1.2-4.6% of all intracranial tumors across all ages
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- 6-11% of all pediatric intracranial tumors
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- Incidence = 0.5-2.5 new cases per 1 million per year
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- ~ 54% of all pediatric sellar/chiasmatic region tumors are CPs
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- ## Natural History & Prognosis
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- Typically slow-growing benign neoplasm
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- Prognosis based upon size, extent of tumor at presentation
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- < 5 cm, recurrence rate: 20%
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- > 5 cm, recurrence rate: 83%
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- Overall 10-year survival: 64-96%
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- ## Treatment
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- Methods of primary treatment
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- Radical surgery = gross total resection
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- Complications = hypothalamic injury, endocrine symptoms, vasa vasorum injury, and pseudoaneurysm
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- Surgery may occur via craniotomy, transnasal, transorbital, or endoscopic routes
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- Less invasive surgery = subtotal resection + radiation therapy
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- Biopsy, cyst drainage, and radiation therapy
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- Treatment for residual or recurrent tumor
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- Surgery, radiation therapy, or cyst aspiration
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- Cyst instillation with intracavitary radioisotopes, bleomycin, or other sclerosing agents
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# DIAGNOSTIC CHECKLIST
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- ## Consider
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- Preoperative ophthalmologic and endocrine evaluations
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- ## Image Interpretation Pearls
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- Use NECT to detect Ca⁺⁺ if MR diagnosis is in question
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- Adamantinomatous CP = 90% rule (90% cystic, calcified, enhancing)
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- Papillary CP is typically solid and primarily adult neoplasm
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