Craniopharyngioma: Difference between revisions

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(Created page with "**Subtypes of craniopharyngioma** Adamantinomatous craniopharyngioma - A craniopharyngioma w/ epithelium that form stellate reticulum, wet karatin and basal pallisades. - Up to 95% of cases of this variant shows CTNNB1 mutations and aberrant nuclear expression of beta- catenin. - Bimodal age distribution: childhood peak age 5–15 years, adult peak age 45–60 years.38 Papillary craniopharyngioma - A craniopharyngioma w/ papillary features. - Occurs in the supratento...")
 
 
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**Subtypes of craniopharyngioma**
* Uncommon, benign (WHO I) tumor that occurs in both children & adults.
= Subtypes of craniopharyngioma =
== Adamantinomatous craniopharyngioma (ACP) ==
* epithelium that form stellate reticulum, wet keratin & basal palisades.
* Up to 95% of this variant shows CTNNB1 mutations & aberrant nuclear expression of β-catenin.
* Bimodal age distribution: <mark>childhood</mark> peak age 5–15 yrs, adult peak age 45–60 years.


Adamantinomatous craniopharyngioma
== Papillary craniopharyngioma ==
* epithelium that form papillary features.
* MC in supratentorial compartment or 3rd ventricle.
* 81–95% of cases show BRAF V600E mutations.
* Usually solid, or rarely cystic.
* almost exclusively in <mark>adults</mark> w/ ~40–55 y/o.


- A craniopharyngioma w/ epithelium that form stellate reticulum, wet karatin and basal pallisades.
= Overview of Adult and Pediatric Craniopharyngioma =
- Up to 95% of cases of this variant shows CTNNB1 mutations and aberrant nuclear expression of beta- catenin.
<table class="wikitable">
- Bimodal age distribution: childhood peak age 5–15 years, adult peak age 45–60 years.38
  <tr>
    <th></th>
    <th>Adult</th>
    <th>Pediatric</th>
  </tr>
  <tr>
    <td><strong>EPIDEMIOLOGY</strong></td>
    <td></td>
    <td></td>
  </tr>
  <tr>
    <td>Age in years</td>
    <td>65–74</td>
    <td>5–14</td>
  </tr>
  <tr>
    <td>Gender</td>
    <td>M = F</td>
    <td>M > F</td>
  </tr>
  <tr>
    <td>Clinical presentation</td>
    <td>Vision loss, hydrocephalus</td>
    <td>Headaches, hydrocephalus</td>
  </tr>
  <tr>
    <td>Endocrine dysfunction</td>
    <td>Less common</td>
    <td>Common</td>
  </tr>
  <tr>
    <td><strong>RADIOLOGY</strong></td>
    <td></td>
    <td></td>
  </tr>
  <tr>
    <td>Cysts</td>
    <td>Less common</td>
    <td>Common</td>
  </tr>
  <tr>
    <td>Calcification</td>
    <td>Less common</td>
    <td>Common</td>
  </tr>
  <tr>
    <td><strong>PATHOLOGY TYPE</strong></td>
    <td></td>
    <td></td>
  </tr>
  <tr>
    <td>Adamantinomatous</td>
    <td>Yes</td>
    <td>Yes</td>
  </tr>
  <tr>
    <td>Papillary</td>
    <td>Yes</td>
    <td>No</td>
  </tr>
  <tr>
    <td><strong>TREATMENT OPTIONS</strong></td>
    <td></td>
    <td></td>
  </tr>
  <tr>
    <td>Surgery</td>
    <td>Yes</td>
    <td>Yes</td>
  </tr>
  <tr>
    <td>Radiotherapy</td>
    <td>Yes</td>
    <td>Yes</td>
  </tr>
  <tr>
    <td>Targeted therapy</td>
    <td>Yes (papillary subtype)</td>
    <td>No</td>
  </tr>
  <tr>
    <td><strong>TREATMENT MORBIDITY</strong></td>
    <td></td>
    <td></td>
  </tr>
  <tr>
    <td>Surgery</td>
    <td></td>
    <td>Better tolerated than radiation</td>
  </tr>
  <tr>
    <td>Radiotherapy</td>
    <td>Better tolerated than surgery</td>
    <td></td>
  </tr>
</table>


Papillary craniopharyngioma
= Genetics =
* ~>95% papillary tumors harbor BRAFV600E mutations → which drive signaling MAP kinase pathway
* ACPs (~94%-96%) activating mutations in exon 3 of CTNNB1 → dysregulation in Wnt/β-catenin signaling


- A craniopharyngioma w/ papillary features.
[[Category:Neuro-Oncology]]
- Occurs in the supratentorial compartment or 3rd ventricle.
[[Category:Extrinsic Brain Tumors]]
- 81–95% of cases show BRAF V600E mutations.
- Usually solid, or rarely cystic.
- Occurs almost exclusively in adults with a mean age of 40–55 years. Epidemiology

Latest revision as of 21:16, 3 March 2024

  • Uncommon, benign (WHO I) tumor that occurs in both children & adults.

Subtypes of craniopharyngioma

Adamantinomatous craniopharyngioma (ACP)

  • epithelium that form stellate reticulum, wet keratin & basal palisades.
  • Up to 95% of this variant shows CTNNB1 mutations & aberrant nuclear expression of β-catenin.
  • Bimodal age distribution: childhood peak age 5–15 yrs, adult peak age 45–60 years.

Papillary craniopharyngioma

  • epithelium that form papillary features.
  • MC in supratentorial compartment or 3rd ventricle.
  • 81–95% of cases show BRAF V600E mutations.
  • Usually solid, or rarely cystic.
  • almost exclusively in adults w/ ~40–55 y/o.

Overview of Adult and Pediatric Craniopharyngioma

Adult Pediatric
EPIDEMIOLOGY
Age in years 65–74 5–14
Gender M = F M > F
Clinical presentation Vision loss, hydrocephalus Headaches, hydrocephalus
Endocrine dysfunction Less common Common
RADIOLOGY
Cysts Less common Common
Calcification Less common Common
PATHOLOGY TYPE
Adamantinomatous Yes Yes
Papillary Yes No
TREATMENT OPTIONS
Surgery Yes Yes
Radiotherapy Yes Yes
Targeted therapy Yes (papillary subtype) No
TREATMENT MORBIDITY
Surgery Better tolerated than radiation
Radiotherapy Better tolerated than surgery

Genetics

  • ~>95% papillary tumors harbor BRAFV600E mutations → which drive signaling MAP kinase pathway
  • ACPs (~94%-96%) activating mutations in exon 3 of CTNNB1 → dysregulation in Wnt/β-catenin signaling
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