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KEY FACTS

Terminology
- Benign, partially cystic sellar region tumor derived from remnants of craniopharyngeal duct/Rathke pouch epithelium
- 2 types - Adamantinomatous (cystic mass in childhood) - Papillary (solid mass in older adults)
Imaging
- General features - Multilobulated, often large (> 5 cm) - Occasionally giant, multicompartmental
- CT: Cystic (90%), Ca⁺⁺ (90%), enhancing (90%)
- MR: Signal varies with cyst contents - Cysts variably hyperintense on T1WI and T2WI - Solid portions enhance heterogeneously; cyst walls enhance strongly - Cyst contents show broad lipid peak (0.9-1.5 ppm) on MR spectroscopy
Pathology
- Most common pediatric intracranial tumor of nonglial origin
- WHO grade 1
Clinical Issues
- Bimodal age distribution - Peak 5-15 years; adults 45-60 years (commonly papillary)
- Pediatric patient with morning headache, visual defect, short stature - Endocrine disturbances include growth hormone (GH) deficiency, luteinizing hormone (LH)/follicle-stimulating hormone (FSH) deficiency - Others = hypothyroidism > adrenal failure > diabetes insipidus
- Surgical resection is primary therapy
- Surgery, radiation therapy, or cyst aspiration for recurrent tumors

TERMINOLOGY

Abbreviations
- Craniopharyngioma (CP)
Synonyms
- Craniopharyngeal duct tumor, Rathke pouch tumor, adamantinoma
Definitions
- Benign, partially cystic sellar region tumor derived from Rathke pouch epithelium - 2 histologies: Adamantinomatous and papillary

IMAGING

General Features

Best diagnostic clue

- CT: Partially Ca⁺⁺ mixed solid/cystic suprasellar mass in child
- MR: Complex signal intensity suprasellar mass

Location

- Surgical division of CPs into 3 groups
        - Sellar
        - Prechiasmatic
        - Retrochiasmatic
- Imaging locations of CPs (adamantinomatous type)
        - Suprasellar (75%)
        - Suprasellar + intrasellar component (21%)
        - Entirely intrasellar (4%)
        - Often extends into multiple cranial fossae: Anterior (30%), middle (23%), posterior, &/or retroclival (20%)
- Rare ectopic locations
        - Optic chiasm, 3rd ventricle
        - Other: Nasopharynx, paranasal sinuses, pineal gland, sphenoid (clivus), cerebellopontine angle

Size

- Variable; often large at presentation (> 5 cm)
        - Occasionally giant, multicompartmental

Morphology
```
- Multilobulated, multicystic
```

CT Findings

NECT

- Adamantinomatous type (90% rule)
        - 90% mixed solid (isodense), cystic (hypodense)
        - 90% calcify
        - 90% enhance (solid = nodule; rim = capsule)
- Papillary type: Often solid, isodense, rarely calcifies

MR Findings

T1WI

- Signal varies with cyst contents
        - Short T1 due to high protein content
        - Classic (adamantinomatous type)
                  - Hyperintense cyst + heterogeneous nodule
        - Less common (papillary type)
                  - Isointense solid component

T2WI

- Cysts are variably hyperintense
- Solid component = heterogeneous (iso-/hyperintense, Ca⁺⁺ portions hypointense)
- Hyperintense signal in brain parenchyma adjacent to tumor may indicate
        - Gliosis, tumor invasion, irritation from leaking cyst fluid
        - Edema from compression of optic chiasm/tracts
        - Hypointense T2* = Ca⁺⁺

FLAIR
```
- Cyst contents typically hyperintense
```

DWI

- Variable depending upon character of cyst fluid

T1WI C+

- Solid portions enhance heterogeneously; cyst walls enhance strongly

MRA

- Vascular displacement &/or encasement

MRS

- Cyst contents show broad lipid spectrum (0.9-1.5 ppm)

Imaging Recommendations

Best imaging tool

- MR with thin sagittal, coronal sequences

Protocol advice

- Pre-/postcontrast T1WI, T2, FLAIR, GRE, DWI, MRS

DIFFERENTIAL DIAGNOSIS

Rathke Cleft Cyst
- Noncalcified, less heterogeneous
- Look for intracystic nodule on T2
- Does not enhance - Claw sign (enhancing pituitary draped around cyst)
- Small Rathke cleft cyst (RCC) may be indistinguishable from rare intrasellar CP
- RCCs express CK8 and CK20 (CPs generally do not)
Suprasellar Arachnoid Cyst
- No Ca⁺⁺, enhancement
Hypothalamic/Chiasmatic Astrocytoma
- Solid or with small cystic/necrotic components
- Ca⁺⁺ is rare; robust enhancement is common
Pituitary Adenoma
- Rare in prepubescent children
- Isointense with brain
- Enhances strongly
- Can mimic CP when cystic and hemorrhagic
Epidermoid/Dermoid Tumors
- Minimal or no enhancement
Thrombosed Aneurysm
- Contains blood products; use SWI
- Look for residual patent lumen, phase artifact
Germinoma or Mixed Germ Cell Tumor With Cystic Component(s)
- Cerebrospinal fluid spread is common, Ca⁺⁺ is rare

PATHOLOGY

General Features

Etiology

- 2 proposed theories
        - CPs arise from remnants of craniopharyngeal duct and Rathke pouch epithelium
        - CPs arise from squamous epithelial cells in pars tuberalis of adenohypophysis

Genetics

- No known genetic susceptibility (rare reports of siblings, parent-child)
- Small subset of CPs are monoclonal tumors that arise from oncogenes at specific loci
- Adamantinomatous: *CTNNB1* mutations and aberrant nuclear expression of β-catenin in up to 95% of cases
- Papillary:*BRAF* V600E mutations in 81-95% of cases

Staging, Grading, & Classification
- WHO grade 1
- MIB-1 labeling index > 7% predicts recurrence
Gross Pathologic & Surgical Features
- Solid tumor with variable cysts
- Adamantinomatous cysts often contain thick "crankcase oil" fluid
- Epithelial fronds penetrate adjacent hypothalamus/chiasm
Microscopic Features
- Adamantinomatous (mostly pediatric) - Multistratified squamous epithelium with nuclear palisading - Nodules of "wet" keratin - Dystrophic Ca⁺⁺
- Papillary (mostly adults) - Sheets of squamous epithelium form pseudopapillae - Villous fibrovascular stroma
- Malignant transformation, distant metastases rare - May occur with varied histologies, resulting in poor prognosis

CLINICAL ISSUES

Presentation

Most common signs/symptoms

- Symptoms vary with location, size of tumor, age of patient
- Visual disturbances (60-85%)
        - Bitemporal hemianopsia

Other signs/symptoms

- Endocrine disturbances (52-87%)
        - Growth hormone (GH) deficiency (75%) > luteinizing hormone (LH)/follicle-stimulating hormone (FSH) deficiency > hypothyroidism > adrenal failure > diabetes insipidus
        - Headaches
        - Cognitive impairment (~ 50%)

Clinical profile

- Pediatric patient with morning headache, visual defect, short stature

Demographics

Age

- Bimodal distribution (peak 5-15 years, with smaller peak 45-60 years)
        - Papillary CP: 40-55 years

Sex
```
- M = F
```
Ethnicity
```
- More common in Japanese children
```

Epidemiology

- Most common pediatric intracranial tumor of nonglial origin
- Comprise 1.2-4.6% of all intracranial tumors across all ages
        - 6-11% of all pediatric intracranial tumors
        - Incidence = 0.5-2.5 new cases per 1 million per year
- ~ 54% of all pediatric sellar/chiasmatic region tumors are CPs

Natural History & Prognosis
- Typically slow-growing benign neoplasm
- Prognosis based upon size, extent of tumor at presentation - < 5 cm, recurrence rate: 20% - > 5 cm, recurrence rate: 83% - Overall 10-year survival: 64-96%
Treatment
- Methods of primary treatment - Radical surgery = gross total resection - Complications = hypothalamic injury, endocrine symptoms, vasa vasorum injury, and pseudoaneurysm - Surgery may occur via craniotomy, transnasal, transorbital, or endoscopic routes - Less invasive surgery = subtotal resection + radiation therapy - Biopsy, cyst drainage, and radiation therapy
- Treatment for residual or recurrent tumor - Surgery, radiation therapy, or cyst aspiration - Cyst instillation with intracavitary radioisotopes, bleomycin, or other sclerosing agents

DIAGNOSTIC CHECKLIST

Consider
- Preoperative ophthalmologic and endocrine evaluations
Image Interpretation Pearls
- Use NECT to detect Ca⁺⁺ if MR diagnosis is in question
- Adamantinomatous CP = 90% rule (90% cystic, calcified, enhancing)
- Papillary CP is typically solid and primarily adult neoplasm

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References

Selected References

Images

Selected Images

Sagittal graphic shows a predominantly cystic, partially solid, suprasellar mass with focal rim Ca⁺⁺. Note the small intrasellar component and fluid-fluid level. Craniopharyngiomas are the 90% tumors (90% cystic, 90% Ca⁺⁺, and 90% enhancing).

Sagittal T2 MR in a 9 year old with headache and visual changes shows a heterogeneous cystic and solid sellar and suprasellar craniopharyngioma with anterior extension into the sphenoid sinus and superior displacement of the optic chiasm.

Coronal T2 MR in a 48-year-old man who presented with visual changes shows a cystic suprasellar mass with a focal T2-hypointense nodule . The T2 imaging mimics a Rathke cleft cyst.

Sagittal T1 C+ MR in a 45-year-old woman shows a cystic and solid mass with an enhancing portion , which distinguishes this cystic lesion as a craniopharyngioma, not a Rathke cleft cyst. Surgical resection is the primary therapy for this WHO grade 1 neoplasm. However, the recurrence rate at 10 years approaches 20%.

Gross pathologic specimen shows a typical solid and cystic composition of an adamantinomatous craniopharyngioma. The cystic spaces contain a thick gelatinous material. (Courtesy AFIP.)

Coronal T2 MR in a 2 year old with a huge suprasellar mass shows multiple hyperintense cysts . Adamantinomatous craniopharyngiomas typically present between 5-15 years in childhood and between 45-60 years in adults. They are the most common pediatric intracranial tumor of nonglial origin.

Axial NECT shows classic findings of an adamantinomatous craniopharyngioma. Note the large suprasellar cyst with a fluid-fluid level , rim , and globular Ca⁺⁺. Enhancement was present on postcontrast images.

Sagittal gross pathology shows classic adamantinomatous craniopharyngioma with mixed solid, cystic components. The classic machine or "crankcase oil" is present. Note the intrasellar extension . (Courtesy R. Hewlett, MD.)

Sagittal T1 C+ MR in a 45-year-old man with visual changes shows a solid enhancing suprasellar mass . Papillary craniopharyngioma was diagnosed at resection. Papillary craniopharyngiomas are WHO grade 1 tumors; however, they are more commonly solid, noncalcified lesions.

Sagittal T1WI C+ MR shows a large recurrent craniopharyngioma in the central skull base and nasopharynx with typical heterogeneous cystic and solid morphology. (Courtesy S. Blaser, MD.)

Additional Images

Axial T1 C+ MR in a child with a huge multilobulated craniopharyngioma shows rim and solid nodular enhancement. Note that the cyst fluid is moderately hyperintense compared to CSF in the lateral ventricles.

Axial DWI MR in the same patient with a large craniopharyngioma shows no restriction in the fluid-containing part of the tumor .

Axial NECT shows a low-attenuation suprasellar mass with rim and globular Ca⁺⁺. Note the fluid-fluid level formed by intracystic keratin debris .

Sagittal T1WI MR shows a complex predominantly cystic suprasellar mass. Note the T1 shortening within the cyst due to machine oil-like proteinaceous fluid .

Axial NECT shows a predominantly solid, minimally calcified , suprasellar craniopharyngioma.

Sagittal T1 C+ MR shows a principally cystic, sellar/suprasellar mass with rim enhancement .

Sagittal T1 C+ MR in a 48-year-old man with visual changes shows an enhancing nodule , which distinguishes this cystic lesion as a craniopharyngioma, not a Rathke cleft cyst. Surgical resection is the primary therapy for this WHO grade 1 neoplasm; however, the recurrence rate at 10 years approaches 20%.

Sagittal T1WI C+ MR shows a complex cystic suprasellar mass with an enhancing rim and solid components . The cysts contain fluid of different signal intensities. Note the large suprasellar, smaller intrasellar components in this classic craniopharyngioma.

A short TE (35) H-MRS in a patient with a solid and cystic craniopharyngioma acquired from the center of the cystic portion of the mass shows large lipid-lactate peaks , characteristic of the cholesterol and lipid constituents found in cysts of craniopharyngiomas.

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KEY FACTS

Terminology

Imaging

Pathology

Clinical Issues

TERMINOLOGY

Abbreviations

Synonyms

Definitions

IMAGING

General Features

Best diagnostic clue

Location

Size

Morphology

CT Findings

NECT

MR Findings

T1WI

T2WI

FLAIR

DWI

T1WI C+

MRA

MRS

Imaging Recommendations

Best imaging tool

Protocol advice

DIFFERENTIAL DIAGNOSIS

Suprasellar Arachnoid Cyst

Hypothalamic/Chiasmatic Astrocytoma

Pituitary Adenoma

Epidermoid/Dermoid Tumors

Thrombosed Aneurysm

PATHOLOGY

General Features

Etiology

Genetics

Staging, Grading, & Classification

Gross Pathologic & Surgical Features

Microscopic Features

CLINICAL ISSUES

Presentation

Most common signs/symptoms

Other signs/symptoms

Clinical profile

Demographics

Age

Sex

Ethnicity

Epidemiology

Natural History & Prognosis

Treatment

DIAGNOSTIC CHECKLIST

Consider

Image Interpretation Pearls

References

Selected References

Images

Selected Images

Additional Images

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