CNS tumor
Sellar region tumors
Papillary craniopharyngioma


Minor changes: 26 May 2021

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PubMed Search: Papillary craniopharyngioma[title]

See Also: Adamantinomatous craniopharyngioma

Angela France, D.O.
Nelli S. Lakis, M.D., M.Sc.
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Cite this page: France A, Lakis NS. Papillary craniopharyngioma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/cnstumorpapcraniopharyngioma.html. Accessed June 20th, 2021.
Definition / general
  • Suprasellar (usually) epithelial encapsulated neoplasm with well differentiated nonkeratinizing squamous epithelium and papillary fibrovascular stroma
  • BRAF V600E mutation in almost all cases
Essential features
  • 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
Sites
  • Suprasellar or intraventricular (third ventricle)
Pathophysiology
Clinical features
  • Visual disturbances (Orphanet J Rare Dis 2007;2:18)
  • Obstructive hydrocephalus
  • Mental / personality changes
  • Hyperprolactinemia (pituitary stalk effect)
  • Diencephalic syndrome (rare)
Diagnosis
  • Lesions usually solid (J Neurosurg 1995;83:206)
  • 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 lacks the prominent cystic component (Childs Nerv Syst 2019;35:169, J Neurosurg 1995;83:206)
  • Solid or contains a few smaller cysts
  • May have cyst and mural nodule configuration
  • Tends 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 cerebrospinal fluid (CSF) density
    • Solid component near soft tissue density
    • Vivid enhancement
    • Calcifications very rare
Radiology images

Contributed by Chunyu "Hunter" Cai, M.D., Ph.D.
T1 precontrast sagittal

T1 precontrast sagittal

T1 precontrast coronal

T1 precontrast coronal



Images hosted on other servers:

Suprasellar mass

Solid suprasellar mass with small cystic component

Preoperative MRI

Prognostic factors
Case reports
Treatment
Gross description
Microscopic (histologic) description
Microscopic (histologic) images

Contributed by Nelli S. Lakis, M.D., M.Sc. and Chunyu "Hunter" Cai, M.D., Ph.D.
Papillary architecture

Papillary architecture

Fibrovascular cores

Fibrovascular cores

Stroma

Stroma

H&E

Lymphocytic inflammation

BRAF V600E immunostain

BRAF V600E immunostain

Virtual slides

Images hosted on other servers:

54 year old man with papillary craniopharyngioma

Cytology description
  • Sheets of epithelial cells
  • Individual nucleated squamous cells
Positive stains
Negative stains
Molecular / cytogenetics description
  • BRAF V600E mutation in 95% of cases
Sample pathology report
  • Sellar / suprasellar region, suprasellar mass, endoscopic resection:
    • Papillary craniopharyngioma (see comment)
    • Comment: BRAF V600E immunostain shows positive cytoplasmic reactivity, supporting / confirming the diagnosis of papillary craniopharyngioma.
Differential diagnosis
  • Adamantinomatous craniopharyngioma:
    • Irregular, infiltrative borders
    • Complex architecture
    • Wet keratin
    • Calcifications
    • Peripheral palisading
    • Loose stellate reticulum
    • Positive nuclear beta catenin in keratin whorls (may be focal)
  • Epidermoid cyst:
    • Uniloculate with thin layer of keratinizing squamous epithelium and keratohyalin granules
  • Rathke cleft cyst with squamous metaplasia:
    • Usually cystic without solid component, squamous epithelium with ciliated or mucus containing cells
Board review style question #1

A 56 year old man is being evaluated due to headaches and visual disturbances. A brain MRI shows a 5 cm solid and cystic suprasellar lesion. The lesion is resected and a histopathologic examination reveals fibrovascular cores and abundant well differentiated squamous epithelium. Which of the following is a feature of the most likely diagnosis?

  1. BRAF V600E mutation
  2. Most commonly occurs in children
  3. Nuclear palisading
  4. Nuclear staining with beta catenin
Board review style answer #1
A. BRAF V600E mutation

Comment Here

Reference: Papillary craniopharyngioma
Board review style question #2
A 43 year old woman is seen in the clinic due to visual disturbances. A physical exam is unremarkable except for visual field testing, which reveals a bitemporal hemianopia. An MRI of the cranium is performed and reveals the presence of a solid and cystic mass in the suprasellar region. The lesion is resected and histologic examination reveals abundant well differentiated squamous epithelium overlying fibrovascular cores, membranous staining of beta catenin and presence of the BRAF V600E mutation. Which of the following is the most likely diagnosis?

  1. Adamantinomatous craniopharyngioma
  2. Dermoid cyst
  3. Papillary craniopharyngioma
  4. Rathke cleft cyst
Board review style answer #2
C. Papillary craniopharyngioma

Comment Here

Reference: Papillary craniopharyngioma
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