Craniopharyngioma

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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.

General

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

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]
  • 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.

Imaging

Radiology:[1]

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

Gross

  • 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.

Images

  • Calcifications in adamantinomatous craniopharyngioma. (WC/Garnett et al.)

  • Autopsy case with papillary craniopharyngioma of the 3rd ventricle. (WC/AFIP)

  • Cholesterol crystals, a typical finding in the cyst fluid, are readily identified by examination under polarized light. (WC/AFIP)

Microscopic

Adamantinomatous

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).

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)

  • Brain infiltration. "Wet" keratin present in two tumor protrusions. The surrounding CNS shows extensive piloid gliosis. (WC/jensflorian)

  • Adamantinomatous Craniopharyngeoma - Trabecular growth pattern. (WC/jensflorian)

  • Adamantinomatous Craniopharyngeoma - Intraoperative frozen section. (WC/jensflorian)

Papillary

Features (papillary):[7]

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

Notes:

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

Images

  • 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)

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Differential diagnosis

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

Trivia

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


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, 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: http://library.med.utah.edu/WebPath/jpeg4/ENDO115.jpg. Accessed on: 6 December 2010.
  9. ↑ URL:[1]. Accessed on: 21 March 2015.
  10. ↑ URL:http://neuropathology-web.org/chapter7/chapter7dMiscellaneous.html]. 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.
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