From Libre Pathology
Jump to navigation Jump to search

Craniopharyngioma is a benign epithelial neuropathology tumour.

Adamantinomatous craniopharyngioma
Diagnosis in short

Adamantinomatous craniopharyngioma. HPS stain.

LM well-circumscribed (or pseudoinvasive border), multicystic, small-to-medium sized cells with moderate amount of basophilic cytoplasm, bland nuclei (with occ. small nucleoli), "wet" keratin (nests of whorled keratin), calcifications
Gross cystic mass filled with motor oil-like fluid
Site sella turcica

Clinical history adults & children
Radiology classically calcified
Prognosis benign
Clin. DDx other sella turcica lesions
Papillary craniopharyngioma
Diagnosis in short

Papillary craniopharyngioma. HPS stain.

LM non-keratinized squamous epithelium (without nuclear atypia), fibrovascular cores (required for papillary)
Site sella turcica

Clinical history adults
Prognosis benign
Clin. DDx other sella turcica lesions

It is subdivided into papillary craniopharyngioma and adamantinomatous craniopharyngioma.


  • Develop from remains of Rathke's pouch or squamous epithelial cell rests.[1]
  • corresponds histologically to WHO grade I.


  • Adamantinomatous type.
  • Squamous papillary type.


  • Adults and children.
  • Typically contain mutations in CTNNB1 (the gene that encodes β-catenin).[2]


  • Adults individuals.[3]
  • Typically contain BRAF V600E mutations.[4]
  • Usually solid.

Clinical features

  • Usu. located in the suprasellar cistern.
    • Rare locations: Cerebellopontine angle, sphenoid sinus, third ventricle.
  • Visual problems.
  • Endocrine deficiencies.
  • Hypothalamic dysfunction (obesity).
  • More frequent in asia than in Europe/US.



  • Calcifications (adamantinous type).
  • Contrast enhancing.
  • Cystic portions.


  • Cystic mass filled with motor oil-like fluid.[5]
    • May not be seen in the papillary variant of craniopharyngioma.
  • Calcified - adamantinomatous type only.
  • Solid & cystic.




Features (adamantinomatous):[6]

  • Trabecular squamous epithelium bordered by palisaded columnar epithelum.
  • Lobules with loosely distributed epithelia ("stellate reticulum").
  • Well-circumscribed (or pseudoinvasive border).
  • Multicystic.
  • Small-to-medium sized cells with moderate amount of basophilic cytoplasm.
  • Bland nuclei (with occ. small nucleoli).
  • "Wet" keratin - nests of whorled keratin.
  • Calcifications (non-psammomatous).



Features (papillary):[7]

  • Non-keratinized squamous epithelium (without nuclear atypia).
  • Fibrovascular cores (required for papillary).


  • +/-Cilia (rare).
  • +/-Goblet cell-like formations (rare).



Differential diagnosis

  • Xanthogranuloma
  • Rathke cyst
  • Epidermoid
  • Well-differentiated carcinoma metastasis


  • The oldest specimen from 1828 is displayed in the pathological-anatomical museum of Vienna.[11]

See also


  1. 1.0 1.1 1.2 Garnett, MR.; Puget, S.; Grill, J.; Sainte-Rose, C. (2007). "Craniopharyngioma.". Orphanet J Rare Dis 2: 18. doi:10.1186/1750-1172-2-18. PMID 17425791.
  2. Preda, V.; Larkin, SJ.; Karavitaki, N.; Ansorge, O.; Grossman, AB. (Oct 2014). "The Wnt Signalling Cascade and the Adherens Junction Complex in Craniopharyngioma Tumorigenesis.". Endocr Pathol. doi:10.1007/s12022-014-9341-8. PMID 25355426.
  3. Giangaspero, F.; Burger, PC.; Osborne, DR.; Stein, RB. (Jan 1984). "Suprasellar papillary squamous epithelioma ("papillary craniopharyngioma").". Am J Surg Pathol 8 (1): 57-64. PMID 6696166.
  4. Brastianos, PK.; Taylor-Weiner, A.; Manley, PE.; Jones, RT.; Dias-Santagata, D.; Thorner, AR.; Lawrence, MS.; Rodriguez, FJ. et al. (Feb 2014). "Exome sequencing identifies BRAF mutations in papillary craniopharyngiomas.". Nat Genet 46 (2): 161-5. doi:10.1038/ng.2868. PMID 24413733.
  5. Fernandez-Miranda, JC.; Gardner, PA.; Snyderman, CH.; Devaney, KO.; Strojan, P.; Suárez, C.; Genden, EM.; Rinaldo, A. et al. (Jul 2012). "Craniopharyngioma: a pathologic, clinical, and surgical review.". Head Neck 34 (7): 1036-44. doi:10.1002/hed.21771. PMID 21584897.
  6. Tadrous, Paul.J. Diagnostic Criteria Handbook in Histopathology: A Surgical Pathology Vade Mecum (1st ed.). Wiley. pp. 184. ISBN 978-0470519035.
  7. Perry, Arie; Brat, Daniel J. (2010). Practical Surgical Neuropathology: A Diagnostic Approach: A Volume in the Pattern Recognition series (1st ed.). Churchill Livingstone. pp. 406. ISBN 978-0443069826.
  8. URL: Accessed on: 6 December 2010.
  9. URL:[1]. Accessed on: 21 March 2015.
  10. URL:]. Accessed on: 21 March 2015.
  11. Pascual, JM.; Prieto, R.; Rosdolsky, M.; Hofecker, V.; Strauss, S.; Winter, E.; Ulrich, W. (Sep 2019). "Joseph Engel (1816-1899), author of a meaningful dissertation on tumors of the pituitary infundibulum: his report on the oldest preserved whole craniopharyngioma specimen.". Virchows Arch. doi:10.1007/s00428-019-02664-z. PMID 31511968.