Craniopharyngioma classification

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Marjan Khan M.B.B.S.[2]

Overview

There are two subtypes of craniopharyngioma based on the histological and imaging features: Adamantinomatous and papillary.

Classification

• Craniopharyngiomas are believed to derive from Rathke cleft rather than squamous cell rests.

• Craniopharyngiomas are classified according to their histologic appearance.
• The histological appearances of the two pathological subtypes are different, accounting for the different imaging features.
• The sub types are said to differ not only in appearances, but also in prognosis and epidemiology.

• Adamantinomatous

Microscopic pathology of Adamantinomatous craniopharyngioma.jpegSource:Wikimedia Commons

• • This type is seen predominantly in children.
  • It consists of reticular epithelial cells which have appearances reminiscent of the enamel pulp of developing teeth.
  • There may be single or multiple cysts filled with thick oily fluid high in protein, blood products, and/or cholesterol, creating the so called "machinery oil".
  • "Wet keratin nodules" are a characteristic histological feature.
  • Calcification is usually present: 90%
  • They are more locally aggressive.
  • It has higher rate of recurrence.
  • Activating beta-catenin gene mutations are found in adamantinomatous tumors.

• Papillary

Microscopic pathology of Papillary Craniopharyngioma Source:Wikimedia Commons

• • The papillary subtype is seen almost exclusively in adults.
  • It is formed of masses of metaplastic squamous cells.
  • "Wet keratin" nodule is absent.
  • Cysts do form, but these are less of a feature, and the tumor is more solid.
  • Calcification is uncommon or even rare.
  • They are less locally aggressive.
  • It has lower rate of recurrence.
  • BRAF V600E mutations are observed in papillary craniopharyngiomas.
• Mixed
  • 15%, but shares imaging features and prognosis similar to adamantinomatous.

References

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