Craniopharyngioma: Difference between revisions
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* Uncommon, benign (WHO I) tumor that occurs in both children & adults. | * Uncommon, benign (WHO I) tumor that occurs in both children & adults. | ||
= Subtypes of craniopharyngioma = | = Subtypes of craniopharyngioma = | ||
== Adamantinomatous craniopharyngioma (ACP) == | |||
== Adamantinomatous craniopharyngioma == | |||
* epithelium that form stellate reticulum, wet keratin & basal palisades. | * epithelium that form stellate reticulum, wet keratin & basal palisades. | ||
* Up to 95% of this variant shows CTNNB1 mutations & aberrant nuclear expression of β-catenin. | * 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. | * Bimodal age distribution: <mark>childhood</mark> peak age 5–15 yrs, adult peak age 45–60 years. | ||
== Papillary craniopharyngioma == | == Papillary craniopharyngioma == | ||
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* 81–95% of cases show BRAF V600E mutations. | * 81–95% of cases show BRAF V600E mutations. | ||
* Usually solid, or rarely cystic. | * Usually solid, or rarely cystic. | ||
* almost exclusively in adults w/ ~40–55 y/o. | * almost exclusively in <mark>adults</mark> w/ ~40–55 y/o. | ||
= Overview of Adult and Pediatric Craniopharyngioma = | = Overview of Adult and Pediatric Craniopharyngioma = | ||
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</tr> | </tr> | ||
</table> | </table> | ||
= 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 | |||
[[Category:Neuro-Oncology]] | [[Category:Neuro-Oncology]] | ||
[[Category:Extrinsic Brain Tumors]] | [[Category:Extrinsic Brain Tumors]] | ||
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