CNS tumor
Sellar region tumors
Craniopharyngioma-papillary
Author: Nelli S. Lakis M.D., M.Sc.
Topic Completed: 1 March 2017
Minor changes: 31 July 2020
Copyright: 2003-2020, 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 | Radiology images | Prognostic factors | Case reports | Treatment | 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. Craniopharyngioma-papillary. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/cnstumorpapcraniopharyngioma.html. Accessed August 6th, 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
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
- 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)
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
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
