CNS tumor
Sellar region tumors
Papillary craniopharyngioma
Deputy Editor-in-Chief: Chunyu "Hunter" Cai, M.D., Ph.D.
Minor changes: 26 May 2021
Copyright: 2003-2021, PathologyOutlines.com, Inc.
PubMed Search: Papillary craniopharyngioma[title]
See Also: Adamantinomatous craniopharyngioma
Table of Contents
Definition / general | Essential features | Terminology | Epidemiology | Sites | Pathophysiology | Clinical features | Diagnosis | Laboratory | Radiology description | Radiology images | Prognostic factors | Case reports | Treatment | Gross description | Microscopic (histologic) description | Microscopic (histologic) images | Virtual slides | Cytology description | Positive stains | Negative stains | Molecular / cytogenetics description | Sample pathology report | Differential diagnosis | Additional references | Board review style question #1 | Board review style answer #1 | Board review style question #2 | Board review style answer #2Cite this page: France A, Lakis NS. Papillary craniopharyngioma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/cnstumorpapcraniopharyngioma.html. Accessed September 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
- Incidence: 10% of all craniopharyngiomas
- Age: almost always adults
- No sex predilection
- Reference: J Neuropathol Exp Neurol 2017;76:126
Sites
- Suprasellar or intraventricular (third ventricle)
Pathophysiology
- Possible transition from Rathke cleft cyst
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 coronal
Images hosted on other servers:
Suprasellar mass
Solid suprasellar mass with small cystic component
Preoperative MRI
Prognostic factors
- Prognosis slightly better than adamantinomatous variant (Neurosurgery 1994;35:1001)
Case reports
- 4 year old girl with BRAF V600E mutation (Childs Nerv Syst 2019;35:169)
- 65 year old man with recurrent papillary craniopharyngioma with BRAF V600E mutation (Acta Neurochir (Wien) 2017;159:2217)
- 80 year old man with mixed type craniopharyngioma (Neuropathology 2012;32:171)
Treatment
- Gross total resection is optimal; smoother surface than adamantinomatous tumors, which facilitates excision (Acta Neurochir (Wien) 2017;159:2217)
- 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 (Acta Neurochir (Wien) 2017;159:2217, J Neurosurg 1995;83:206)
- 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 (J Neuropathol Exp Neurol 2017;76:126, J Neurosurg 1995;83:206)
- Crude papillae around fibrovascular cores
- Small collagenous whorls
Microscopic (histologic) images
Contributed by Nelli S. Lakis, M.D., M.Sc. and Chunyu "Hunter" Cai, M.D., Ph.D.
Papillary architecture
Fibrovascular cores
Stroma
Lymphocytic inflammation
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
- CK7, EMA (J Neuropathol Exp Neurol 2017;76:126)
- BRAF VE1 parallels presence of BRAF V600E mutation in 95% of cases
- Can have scant PAS positive goblet cells
- Only membranous beta catenin; nuclei and cytoplasm negative
- Reference: Neuropathol Appl Neurobiol 2015;41:733
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
Additional references
- Clin Neuropathol 2003;22:229, J Egypt Natl Canc Inst 2015;27:139, J Korean Neurosurg Soc 2017;60:108, Acta Neuropathol Commun 2016;4:20, AJNR Am J Neuroradiol 2015;36:E55, eMedicine: Surgery for Craniopharyngiomas Treatment & Management [Accessed 30 April 2021], Radiopaedia: Craniopharyngioma [Accessed 30 April 2021], Radiopaedia: Craniopharyngioma (papillary) [Accessed 30 April 2021], Kleinschmidt-DeMasters: Diagnostic Pathology - Neuropathology, 2nd Edition, 2016, Louis: WHO Classification of Tumours of the Central Nervous System, 4th Edition, 2016
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?
- BRAF V600E mutation
- Most commonly occurs in children
- Nuclear palisading
- Nuclear staining with beta catenin
Board review style answer #1
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?
- Adamantinomatous craniopharyngioma
- Dermoid cyst
- Papillary craniopharyngioma
- Rathke cleft cyst
Board review style answer #2
