--- title: "Craniopharyngioma" docid: "00e66680-6731-4287-b5a1-3f0b3f09053b" authors: - key: "8d5254e9-8dda-478b-8f08-bdee97a32c79" value: "Karen L. Salzman, MD, FACR" breadcrumbs: - name: "Brain" slug: "brain" treeNodeId: "6d8829f1-14d7-45af-8675-255189aa526a" - name: "Diagnosis" slug: "diagnosis" treeNodeId: "51c00394-446e-4a38-94af-d3b1d14d34e8" - name: "Anatomy-Based Diagnoses" slug: "anatomy-based-diagnoses" treeNodeId: "529d3e33-f508-498c-bc70-cf962e81e629" - name: "Sella and Pituitary" slug: "sella-and-pituitary" treeNodeId: "9afaeeb6-661c-49be-b55f-5bdc1c98a53e" - name: "Neoplasms" slug: "neoplasms" treeNodeId: "87c53ba9-d217-4dc9-be90-15cfe4d7766c" - name: "Craniopharyngioma" slug: "craniopharyngioma" treeNodeId: null category: "Brain" documentVersionId: "fba662fa-55e8-45a2-8081-e59d71a73b51" imageCount: 19 lastUpdated: "08/10/20" pageDescription: "Craniopharyngioma" pageKeywords: "Brain, Diagnosis, Anatomy-Based Diagnoses, Sella and Pituitary, Neoplasms, Craniopharyngioma" pageTitle: "Craniopharyngioma | STATdx" enhancedTitle: "Craniopharyngioma" type: "DX" references: true breadcrumbs: - "Brain" - "Diagnosis" - "Anatomy-Based Diagnoses" - "Sella and Pituitary" - "Neoplasms" - "Craniopharyngioma" --- # 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](/document/rathke-cleft-cyst/8f1561f7-92a7-485c-a0ae-2e2d5c8c1628) - 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)](/document/germinoma/078b68a2-67de-457e-818a-63655cec95aa) - 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 c497473c-5835-4221-bfa1-0d2be04bee73 ## References # Selected References 1. [Azuma M et al: Usefulness of contrast-enhanced 3D-FLAIR MR imaging for differentiating Rathke cleft cyst from cystic craniopharyngioma. AJNR Am J Neuroradiol. 41(1):106-10, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=31857323%5Bpmid%5D) 1. [Fouda MA et al: Sixty years single institutional experience with pediatric craniopharyngioma: between the past and the future. Childs Nerv Syst. 36(2):291-6, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=31292757%5Bpmid%5D) 1. [Goldman S et al: Phase II study of peginterferon alpha-2b for patients with unresectable or recurrent craniopharyngiomas: a Pediatric Brain Tumor Consortium report. Neuro Oncol. ePub, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32393959%5Bpmid%5D) 1. [Prince E et al: Transcriptional analyses of adult and pediatric adamantinomatous craniopharyngioma reveals similar expression signatures regarding potential therapeutic targets. 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[Marcus HJ et al: Craniopharyngioma in children: trends from a third consecutive single-center cohort study. J Neurosurg Pediatr. 1-9, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31860822%5Bpmid%5D) 1. [Whelan R et al: Interrater reliability of a method to assess hypothalamic involvement in pediatric adamantinomatous craniopharyngioma. J Neurosurg Pediatr. 1-6, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31604324%5Bpmid%5D) 1. Buslei et al: Craniopharyngioma. In Louis DN et al: WHO Classification of Tumors of the Central Nervous System. IARC. 324-8, 2016 1. [Greenfield BJ et al: Long-term disease control and toxicity outcomes following surgery and intensity modulated radiation therapy (IMRT) in pediatric craniopharyngioma. Radiother Oncol. 114(2):224-9, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25542650%5Bpmid%5D) 1. [Kim JH et al: BRAF V600E mutation is a useful marker for differentiating Rathke's cleft cyst with squamous metaplasia from papillary craniopharyngioma. J Neurooncol. 123(1):189-91, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25820214%5Bpmid%5D) 1. [Lee HJ et al: Pretreatment diagnosis of suprasellar papillary craniopharyngioma and germ cell tumors of adult patients. AJNR Am J Neuroradiol. 36(3):508-17, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25339645%5Bpmid%5D) 1. [Sterkenburg AS et al: Survival, hypothalamic obesity, and neuropsychological/psychosocial status after childhood-onset craniopharyngioma: newly reported long-term outcomes. Neuro Oncol. 17(7):1029-38, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25838139%5Bpmid%5D) 1. [Lee CC et al: Gamma Knife surgery for craniopharyngioma: report on a 20-year experience. J Neurosurg. 121 Suppl:167-78, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=25434950%5Bpmid%5D) 1. [Clark AJ et al: A systematic review of the results of surgery and radiotherapy on tumor control for pediatric craniopharyngioma. Childs Nerv Syst. 29(2):231-8, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=23089933%5Bpmid%5D) 1. [Müller HL: Childhood craniopharyngioma. Pituitary. 16(1):56-67, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=22678820%5Bpmid%5D) 1. [Chentli F et al: Congenital craniopharyngioma: a case report and literature review. J Pediatr Endocrinol Metab. 25(11-12):1181-3, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=23329768%5Bpmid%5D) 1. [Clark AJ et al: Treatment-related morbidity and the management of pediatric craniopharyngioma: a systematic review. J Neurosurg Pediatr. 10(4):293-301, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=22920295%5Bpmid%5D) 1. [İnci MF et al: A rare presentation of craniopharyngioma: delayed puberty. BMJ Case Rep. 2012, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=23195827%5Bpmid%5D) 1. [Shi Z et al: Transient enlargement of craniopharyngioma after radiation therapy: pattern of magnetic resonance imaging response following radiation. J Neurooncol. 109(2):349-55, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=22692563%5Bpmid%5D) 1. [Boongird A et al: Malignant craniopharyngioma; case report and review of the literature. Neuropathology. 29(5):591-6, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19077042%5Bpmid%5D) 1. [Frangou EM et al: Metastatic craniopharyngioma: case report and literature review. Childs Nerv Syst. 25(9):1143-7, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19517118%5Bpmid%5D) 1. [Keil MF et al: Pituitary tumors in childhood: update of diagnosis, treatment and molecular genetics. Expert Rev Neurother. 8(4):563-74, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18416659%5Bpmid%5D) 1. [Garrè ML et al: Craniopharyngioma: modern concepts in pathogenesis and treatment. Curr Opin Pediatr. 19(4):471-9, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=17630614%5Bpmid%5D) 1. [Powers CJ et al: Cerebellopontine angle craniopharyngioma: case report and literature review. Pediatr Neurosurg. 43(2):158-63, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=17337933%5Bpmid%5D) 1. [Rodriguez FJ et al: The spectrum of malignancy in craniopharyngioma. Am J Surg Pathol. 31(7):1020-8, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=17592268%5Bpmid%5D) 1. [Shuman AG et al: Extracranial nasopharyngeal craniopharyngioma: case report. Neurosurgery. 60(4):E780-1; discussion E781, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=17415187%5Bpmid%5D) 1. [Aquilina K et al: Primary cerebellopontine angle craniopharyngioma in a patient with gardner syndrome. Case report and review of the literature. J Neurosurg. 105(2):330-3, 2006](http://www.ncbi.nlm.nih.gov/pubmed/?term=17219843%5Bpmid%5D) 1. [Haupt R et al: Epidemiological aspects of craniopharyngioma. J Pediatr Endocrinol Metab. 19 Suppl 1:289-93, 2006](http://www.ncbi.nlm.nih.gov/pubmed/?term=16700303%5Bpmid%5D) 1. [Prabhu VC et al: The pathogenesis of craniopharyngiomas. Childs Nerv Syst. 21(8-9):622-7, 2005](http://www.ncbi.nlm.nih.gov/pubmed/?term=15965669%5Bpmid%5D) 1. [Wang KC et al: Origin of craniopharyngiomas: implication on the growth pattern. Childs Nerv Syst. 21(8-9):628-34, 2005](http://www.ncbi.nlm.nih.gov/pubmed/?term=16059733%5Bpmid%5D) 1. [Srinivasan S et al: Features of the metabolic syndrome after childhood craniopharyngioma. J Clin Endocrinol Metab. 89(1):81-6, 2004](http://www.ncbi.nlm.nih.gov/pubmed/?term=14715831%5Bpmid%5D) 1. [Behari S et al: Intrinsic third ventricular craniopharyngiomas: report on six cases and a review of the literature. Surg Neurol. 60(3):245-52; discussion 252-3, 2003](http://www.ncbi.nlm.nih.gov/pubmed/?term=12922045%5Bpmid%5D) 1. [Saeki N et al: MR imaging study of edema-like change along the optic tract in patients with pituitary region tumors. AJNR Am J Neuroradiol. 24(3):336-42, 2003](http://www.ncbi.nlm.nih.gov/pubmed/?term=12637278%5Bpmid%5D) 1. [Barajas MA et al: Multimodal management of craniopharyngiomas: neuroendoscopy, microsurgery, and radiosurgery. J Neurosurg. 97(5 Suppl):607-9, 2002](http://www.ncbi.nlm.nih.gov/pubmed/?term=12507105%5Bpmid%5D) 1. [Fujimoto Y et al: Craniopharyngioma involving the infrasellar region: a case report and review of the literature. Pediatr Neurosurg. 37(4):210-6, 2002](http://www.ncbi.nlm.nih.gov/pubmed/?term=12372916%5Bpmid%5D) 1. [Green AL et al: Craniopharyngioma in a mother and daughter. Acta Neurochir (Wien). 144(4):403-4, 2002](http://www.ncbi.nlm.nih.gov/pubmed/?term=12021891%5Bpmid%5D) 1. [Sekine S et al: Craniopharyngiomas of adamantinomatous type harbor beta-catenin gene mutations. Am J Pathol. 161(6):1997-2001, 2002](http://www.ncbi.nlm.nih.gov/pubmed/?term=12466115%5Bpmid%5D) 1. [Van Effenterre R et al: Craniopharyngioma in adults and children: a study of 122 surgical cases. J Neurosurg. 97(1):3-11, 2002](http://www.ncbi.nlm.nih.gov/pubmed/?term=12134929%5Bpmid%5D) 1. [Chen CJ: Suprasellar and infrasellar craniopharyngioma with a persistent craniopharyngeal canal: case report and review of the literature. Neuroradiology. 43(9):760-2, 2001](http://www.ncbi.nlm.nih.gov/pubmed/?term=11594427%5Bpmid%5D) ## 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).](images/app.statdx.com_image_thumbnail_f94f79c3-abc9-4850-a981-c09355881668_size_168_quality_85_a65f69d1_20251014T205219Z.jpg) *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 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).](images/app.statdx.com_image_thumbnail_f94f79c3-abc9-4850-a981-c09355881668_size_174_quality_85_f5f4f66b_20251014T205217Z.jpg) *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.](images/app.statdx.com_image_thumbnail_ff36ded9-e912-42b4-929c-ef63a5e8fa57_size_168_quality_85_f43afdfe_20251014T205219Z.jpg) *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.](images/app.statdx.com_image_thumbnail_a46de544-a40b-46b9-bd8e-dfba34730496_size_168_quality_85_27db027b_20251014T205219Z.jpg) *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%.](images/app.statdx.com_image_thumbnail_3db34480-5109-41cc-8a67-ce27096172de_size_168_quality_85_2a792ca4_20251014T205219Z.jpg) *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.)](images/app.statdx.com_image_thumbnail_8fe443b2-0b89-48cc-a021-94c248ae56c4_size_168_quality_85_275ebdf6_20251014T205219Z.jpg) *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.](images/app.statdx.com_image_thumbnail_7e7095db-3e50-4824-a7f2-b2d59382d174_size_168_quality_85_dd1d82dd_20251014T205220Z.jpg) *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.](images/app.statdx.com_image_thumbnail_20fed2a7-ce3d-4ef8-aab8-c4cf436a651f_size_168_quality_85_f9740012_20251014T205220Z.jpg) *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.)](images/app.statdx.com_image_thumbnail_5737449d-e941-4480-bb6c-7a8d8fc2f41a_size_168_quality_85_6ada1f4b_20251014T205219Z.jpg) *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.](images/app.statdx.com_image_thumbnail_1a1382d8-4859-4020-bd60-8f778d681863_size_168_quality_85_bb86662d_20251014T205219Z.jpg) *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.)](images/app.statdx.com_image_thumbnail_e2df5ace-0993-4081-9e77-4d8541d3bd6a_size_168_quality_85_c4a419f8_20251014T205219Z.jpg) *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.](images/app.statdx.com_image_thumbnail_97cdf507-1f5d-43cc-9886-65f26e0655fb_size_168_quality_85_3525f13e_20251014T205219Z.jpg) *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 .](images/app.statdx.com_image_thumbnail_12002415-4879-47aa-9a98-a0097e492049_size_168_quality_85_a3ee5d4f_20251014T205219Z.jpg) *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 .](images/app.statdx.com_image_thumbnail_ee1a8e06-2a41-47f1-b559-ea7a6c233786_size_168_quality_85_d549a7de_20251014T205219Z.jpg) *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 .](images/app.statdx.com_image_thumbnail_b5c10b79-9905-45ca-969b-3ecbb2003ac1_size_168_quality_85_39e04a96_20251014T205219Z.jpg) *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.](images/app.statdx.com_image_thumbnail_7b7888df-3246-4226-a063-e277639bdc87_size_168_quality_85_2370a8d3_20251014T205219Z.jpg) *Axial NECT shows a predominantly solid, minimally calcified , suprasellar craniopharyngioma.* ![Sagittal T1 C+ MR shows a principally cystic, sellar/suprasellar mass with rim enhancement .](images/app.statdx.com_image_thumbnail_e8b015d3-9981-4dc9-ac0f-f3c80b3eda85_size_168_quality_85_d77cca56_20251014T205219Z.jpg) *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%.](images/app.statdx.com_image_thumbnail_03c2e4a8-4ffe-4f84-975e-88dd1180d675_size_168_quality_85_c18501f8_20251014T205219Z.jpg) *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.](images/app.statdx.com_image_thumbnail_e7b6d109-2017-437b-9268-f9d51f5b4e9d_size_168_quality_85_26288ca2_20251014T205219Z.jpg) *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.](images/app.statdx.com_image_thumbnail_4d9c1527-9689-4e7b-b956-6ea22b77a13d_size_168_quality_85_d0f20fb4_20251014T205219Z.jpg) *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.*