CNS tumor
Tumors of the sellar region
Papillary craniopharyngioma

Author: Nelli S. Lakis, M.D., M.Sc. (see Authors page)

Revised: 29 March 2017, last major update March 2017

Copyright: (c) 2003-2017, PathologyOutlines.com, Inc.

PubMed Search: Papillary craniopharyngioma[title]

Related topics: Adamantinomatous craniopharyngioma
Cite this page: Papillary craniopharyngioma. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/cnstumorpapcraniopharyngioma.html. Accessed April 28th, 2017.
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
Prognostic factors
Case reports
Treatment
Clinical images

Images hosted on other servers:

Missing Image

Contrast enhanced CT demonstrates a suprasellar mass which vividly enhances.
Although contrast does make it harder to assess, there is no convincing evidence
of calcification. The pituitary fossa is unremarkable and the anterior cerebral arteries,
although contacted, are not encased. There is no cystic component.


Missing Image

MRI of the brain demonstrates a predominantly solid enhancing suprasellar
mass with a small cystic component posteriorly, towards the left, which
demonstrates no T1 hyperintensity.

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

Images hosted on Pathout server:

Missing Image

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



Images hosted on other servers:

Missing Image

(H&E) staining of adamantinomatous
and papillary craniopharyngiomas

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