Craniopharyngioma classification
|
Craniopharyngioma Microchapters |
|
Diagnosis |
|---|
|
Treatment |
|
Case Studies |
|
Craniopharyngioma classification On the Web |
|
American Roentgen Ray Society Images of Craniopharyngioma classification |
|
Risk calculators and risk factors for Craniopharyngioma classification |
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. [1]
- 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.[2]
- Adamantinomatous
 |
- This type is seen predominantly in children.[1]
- 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".[3]
- "Wet keratin nodules" are a characteristic histological feature.
- Calcification is usually present in 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.[1]
- 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.[3]
- 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
- ↑ 1.0 1.1 1.2 Mortini P (August 2017). "Craniopharyngiomas: a life-changing tumor". Endocrine. 57 (2): 191–192. doi:10.1007/s12020-016-1192-2. PMID 27981519.
- ↑ Classification of Craniopharyngioma. Cancer gov. http://www.cancer.gov/types/brain/hp/child-cranio-treatment-pdq#link/_40_toc
- ↑ 3.0 3.1 Müller HL (April 2017). "Risk-adapted, long-term management in childhood-onset craniopharyngioma". Pituitary. 20 (2): 267–281. doi:10.1007/s11102-016-0751-0. PMID 27604996.