Craniopharyngioma

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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

Craniopharyngioma is a benign neuropathology tumour.

It is subdivided into papillary craniopharyngioma and adamantinomatous craniopharyngioma.

General

  • Develop from remains of Rathke's pouch or squamous epithelial cell rests.[1]

Subtypes:[1]

  • Adamantinomatous type.
  • Squamous papillary type.

Adamantinomatous

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

Papillary

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

Gross

  • Cystic mass filled with motor oil-like fluid.[5]
    • May not be seen in the papillary variant of craniopharyngioma.

Radiology:[1]

  • Calcified - adamantinomatous type only.
  • Solid & cystic.

Microscopic

Adamantinomatous

Features (adamantinomatous):[6]

  • 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).

Images

  • Adamantinomatous craniopharyngioma - very low mag. (WC/Nephron)

  • Adamantinomatous craniopharyngioma - intermed. mag. (WC/Nephron)

  • Adamantinomatous craniopharyngioma - very high mag. (WC/Nephron)

  • Adamantinomatous Craniopharyngeoma - low power (SKB)

  • Adamantinomatous Craniopharyngeoma - low power (SKB)

  • Adamantinomatous Craniopharyngeoma - low power (SKB)

  • Adamantinomatous Craniopharyngeoma - medium power (SKB)

  • Adamantinomatous Craniopharyngeoma - medium power (SKB)

  • Adamantinomatous Craniopharyngeoma - medium power (SKB)

  • Adamantinomatous Craniopharyngeoma - medium power (SKB)

  • Adamantinomatous Craniopharyngeoma - medium power (SKB)

Papillary

Features (papillary):[7]

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

Notes:

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

Image

  • Papillary craniopharyngioma - intermed. mag. (WC/Nephron)

  • Papillary craniopharyngioma - high mag. (WC/Nephron)

  • Papillary craniopharyngioma - very high mag. (WC/Nephron)

  • Papillary Craniopharyngeoma - low power (SKB)

  • Papillary Craniopharyngeoma - low power (SKB)

  • Papillary Craniopharyngeoma - low power (SKB)

  • Papillary Craniopharyngeoma - medium power (SKB)

  • Papillary Craniopharyngeoma - medium power (SKB)

www:

See also

References

  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 et al. Nat. Genet 2014 doi:10.1038/ng.2868
  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: http://library.med.utah.edu/WebPath/jpeg4/ENDO115.jpg. Accessed on: 6 December 2010.
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