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 along the craniopharyngeal duct as was previously thought. Craniopharyngiomas are classified according to their histologic appearance. The histological appearances of the two pathological subtypes are different, accounting for the different imaging features.These 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
  • 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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