CNS tumor
Sellar region tumors
Craniopharyngioma-papillary


Topic Completed: 1 March 2017

Minor changes: 29 September 2020

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PubMed Search: Papillary craniopharyngioma[title]

Related topics: Adamantinomatous craniopharyngioma

Nelli S. Lakis M.D., M.Sc.
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Cite this page: Lakis NS. Craniopharyngioma-papillary. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/cnstumorpapcraniopharyngioma.html. Accessed October 20th, 2020.
Definition / general
  • Papillary craniopharyngioma: suprasellar (usually) epithelial neoplasm with BRAF V600E mutation in almost all cases
Essential features
  • Always WHO Grade I
  • Encapsulated tumor with well differentiated nonkeratinizing squamous epithelium and papillary fibrovascular stroma
Terminology
  • Suprasellar papillary squamous epithelioma; ciliated craniopharyngioma; ciliated and goblet cell craniopharyngioma
Epidemiology
  • Incidence: 10% of all craniopharyngiomas
  • Age: Almost always adults
  • No sex predilection
Sites
  • Suprasellar or intraventricular (third ventricle)
Etiology
Clinical features
  • Visual disturbances
  • Obstructive hydrocephalus
  • Mental / personality changes
  • Hyperprolactinemia (pituitary stalk effect)
  • Diencephalic syndrome (rare)
Diagnosis
  • Lesions usually solid
  • If cystic, may have mural nodule
  • NO calcifications on CT scan
  • MRI: Contrast enhancing solid or cystic mass
Laboratory
  • Full pituitary endocrine workup is usually mandatory
  • Visual acuity and visual field assessment is also performed to show any deficits and rule out papilledema
Radiology description
  • More spherical in outline and usually lack the prominent cystic component
  • Solid or contain a few smaller cysts
  • May have cyst and mural nodule configuration
  • Tend to displace adjacent structures
  • MRI:
    • T1 weighted images: 85% of cysts are hypointense
    • T1 weighted images: solid component iso- to hypointense
    • Vividly contrast enhancing
  • CT:
    • Cysts small and insignificant
    • Near CSF density
    • Solid component near soft tissue density
    • Vivid enhancement
    • Calcifications very rare
Radiology images

Images hosted on other servers:

Case 1: Contrast enhanced CT demonstrates a suprasellar mass

Case 2: MRI of the brain demonstrates predominantly solid suprasellar mass with small cystic component posteriorly

Prognostic factors
Case reports
Treatment
Gross description
  • Discrete, encapsulated mass
  • Not densely adherent to adjacent brain
  • No cholesterol rich, thick, oily cyst contents
  • If cystic, contains clear liquid
Microscopic (histologic) description
  • Low power highlights papillary configuration with cauliflower-like morphology
  • Solid sheets of well differentiated nonkeratinizing squamous epithelium
  • Crude papillae around fibrovascular cores
  • Small collagenous whorls
Microscopic (histologic) images

Contributed by Nelli S. Lakis, M.D., M.Sc.

Various images

Cytology description
  • Sheets of epithelial cells
  • Individual nucleated squames
Positive stains
  • CK7, EMA
  • BRAF VE1 parallels presence of BRAF V600E mutation in 95% of cases
    • Can have scant PAS positive goblet cells
  • Only membranous β-catenin; nuclei and cytoplasm negative
Negative stains
Molecular / cytogenetics description
  • BRAF V600E mutation in 95% of cases
Differential diagnosis
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Image 01 Image 02