.
This commit is contained in:
Ross
@@ -0,0 +1,476 @@
|
||||
---
|
||||
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. Acta Neuropathol Commun. 8(1):68, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32404202%5Bpmid%5D)
|
||||
1. [Sadashivam S et al: Adult craniopharyngioma: the role of extent of resection in tumor recurrence and long-term functional outcome. Clin Neurol Neurosurg. 192:105711, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32036264%5Bpmid%5D)
|
||||
1. [Soldozy S et al: Endoscopic endonasal surgery outcomes for pediatric craniopharyngioma: a systematic review. Neurosurg Focus. 48(1):E6, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=31896083%5Bpmid%5D)
|
||||
1. [Drapeau A et al: Pediatric craniopharyngioma. Childs Nerv Syst. 35(11):2133-45, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31385085%5Bpmid%5D)
|
||||
1. [Madsen PJ et al: Endoscopic endonasal resection versus open surgery for pediatric craniopharyngioma: comparison of outcomes and complications. J Neurosurg Pediatr. 1-10, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31174192%5Bpmid%5D)
|
||||
1. [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).*
|
||||
|
||||
|
||||
*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 <img src='img/arrows/CO.png'/> with anterior extension into the sphenoid sinus <img src='img/arrows/WS.png'/> 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 <img src='img/arrows/WO.png'/> with a focal T2-hypointense nodule <img src='img/arrows/CS.png'/>. 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 <img src='img/arrows/CO.png'/>, 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 <img src='img/arrows/BO.png'/> 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 <img src='img/arrows/CS.png'/>. 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 <img src='img/arrows/WO.png'/>, rim <img src='img/arrows/CC.png'/>, and globular <img src='img/arrows/CS.png'/> 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" <img src='img/arrows/WO.png'/> is present. Note the intrasellar extension <img src='img/arrows/WC.png'/>. (Courtesy R. Hewlett, MD.)*
|
||||
|
||||
|
||||
*Sagittal T1 C+ MR in a 45-year-old man with visual changes shows a solid enhancing suprasellar mass <img src='img/arrows/WS.png'/>. 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 <img src='img/arrows/WS.png'/> 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 <img src='img/arrows/WS.png'/> and solid nodular <img src='img/arrows/WO.png'/> 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 <img src='img/arrows/WS.png'/>.*
|
||||
|
||||
|
||||
*Axial NECT shows a low-attenuation suprasellar mass with rim <img src='img/arrows/WS.png'/> and globular <img src='img/arrows/WC.png'/> Ca⁺⁺. Note the fluid-fluid level formed by intracystic keratin debris <img src='img/arrows/WO.png'/>.*
|
||||
|
||||
|
||||
*Sagittal T1WI MR shows a complex predominantly cystic suprasellar mass. Note the T1 shortening within the cyst due to machine oil-like proteinaceous fluid <img src='img/arrows/WS.png'/>.*
|
||||
|
||||
|
||||
*Axial NECT shows a predominantly solid, minimally calcified <img src='img/arrows/WS.png'/>, suprasellar craniopharyngioma.*
|
||||
|
||||
|
||||
*Sagittal T1 C+ MR shows a principally cystic, sellar/suprasellar mass with rim enhancement <img src='img/arrows/WS.png'/>.*
|
||||
|
||||
|
||||
*Sagittal T1 C+ MR in a 48-year-old man with visual changes shows an enhancing nodule <img src='img/arrows/WO.png'/>, 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 <img src='img/arrows/WC.png'/> and solid components <img src='img/arrows/WO.png'/>. The cysts contain fluid of different signal intensities. Note the large suprasellar, smaller intrasellar <img src='img/arrows/WS.png'/> 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 <img src='img/arrows/WS.png'/>, characteristic of the cholesterol and lipid constituents found in cysts of craniopharyngiomas.*
|
||||
|
||||
Reference in New Issue
Block a user