CNS tumor
Tumors of the sellar region
Papillary craniopharyngioma
Revised: 2 January 2019, last major update March 2017
Copyright: (c) 2003-2017, PathologyOutlines.com, Inc.
PubMed Search: Papillary craniopharyngioma[title]
Related topics: Adamantinomatous craniopharyngioma
Tumors of the sellar region
Papillary craniopharyngioma
Author: Nelli S. Lakis M.D., M.Sc.
Topic Completed: 1 March 2017
Revised: 2 January 2019, last major update March 2017
Copyright: (c) 2003-2017, PathologyOutlines.com, Inc.
PubMed Search: Papillary craniopharyngioma[title]
Related topics: Adamantinomatous craniopharyngioma
Table of Contents
Definition / general | Essential features | Terminology | Epidemiology | Sites | Etiology | Clinical features | Diagnosis | Laboratory | Radiology description | Prognostic factors | Case reports | Treatment | Clinical images | Gross description | Microscopic (histologic) description | Microscopic (histologic) images | Cytology description | Positive stains | Negative stains | Molecular / cytogenetics description | Differential diagnosis | Additional referencesCite this page: Lakis N. Papillary craniopharyngioma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/cnstumorpapcraniopharyngioma.html. Accessed May 24th, 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
- Possible transition from Rathke cleft cyst
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
- Prognosis slightly better than adamantinomatous variant (Surg Neurol 1992;38:294)
Case reports
- Description of two purely intraventricular craniopharyngiomas with papillary features (Surg Neurol 1992;38:294)
Treatment
- Gross total resection is optimal; smoother surface than adamantinomatous tumors, which facilitates excision
- Some reports show dramatic shrinkage of papillary craniopharyngioma using anti BRAF agents (dabrafenib and trametinib) (Pituitary 2016;19:544, Eur J Endocrinol 2016;174:R139, Neurosurg Focus 2016;41:E3)
Clinical images
Images hosted on other servers:
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.
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:
Contributed by Nelli S. Lakis, M.D., M.Sc.
Images hosted on other servers:
(H&E) staining of adamantinomatous
and papillary craniopharyngiomas
Cytology description
- Sheets of epithelial cells
- Individual nucleated squames
Positive stains
Molecular / cytogenetics description
- BRAF V600E mutation in 95% of cases
Differential diagnosis
- Adamantinomatous craniopharyngioma:
- Irregular, infiltrative borders
- Complex architecture
- "Wet" keratin
- Calcifications
- Peripheral palisading
- Loose "stellate reticulum"
- Positive nuclear β-catenin in keratin whorls
- 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
Additional references
- Clin Neuropathol 2003;22:229, J Egypt Natl Canc Inst 2015;27:139, J Neuropathol Exp Neurol 2017 Jan 9 [Epub ahead of print], J Korean Neurosurg Soc 2017;60:108, Acta Neuropathol Commun 2016;4:20, AJNR Am J Neuroradiol 2015;36:E55, Neuropathol Appl Neurobiol 2015;41:733, eMedicine - Surgery for Craniopharyngiomas Treatment & Management, Radiopaedia.org - Craniopharyngioma, Radiopaedia.org - Craniopharyngioma (papillary), Kleinschmidt-DeMasters, Tihan, Rodriguez: Diagnostic Pathology: Neuropathology, 2nd Edition, 2016
