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 epithelial 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]
  • 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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