Craniopharyngioma
Background
What is the origin of craniopharyngioma?
Epithelial tumor arising from an incompletely involuted hypophyseal-pharyngeal duct (Rathke pouch) from an anatomic region called the tuber cinerium
In what region of the brain does it usually arise?
Suprasellar region (most common), sella proper (less common)
Are craniopharyngiomas malignant?
No. They are histologically benign but are problematic due to local progression around critical structures.
Approximately how many cases of craniopharyngioma occur annually in the U.S.?
~300–350 cases/yr of craniopharyngioma in the U.S.
At what ages does craniopharyngioma occur?
Median age 5–10 yrs; higher risk age <20 yrs and >40 yrs (bimodal); one third of cases occur in pts age 0–14 yrs.
What are the 2 histologic subtypes of craniopharyngioma?
Adamantinomatous and squamous
Which subtype is characterized by a solid and cystic pattern?
Adamantinomatous craniopharyngioma has a solid and cystic pattern.
Historically, how has the cyst fluid consistency been described?
“Machine oil” like (very proteinaceous fluid)
What structures do cysts usually abut superiorly?
Tumors/cysts usually abut the 3rd ventricle and the hypothalamus superiorly.
Name the most common presenting signs/Sx of craniopharyngioma.
HA, N/V (i.e., ↑ICP), visual change, diabetes insipidus
What is the most common hormone deficiency at presentation?
At presentation, GH is the most common hormone deficiency.
Which type of craniopharyngioma has a better prognosis: calcified or noncalcified?
Noncalcified craniopharyngioma has a better prognosis.
Do craniopharyngioma tumors respond rapidly or slowly to RT?
Craniopharyngioma tumors respond very slowly to RT.
Workup/Staging
What is the workup for a craniopharyngioma?
Craniopharyngioma workup: H&P, basic labs, pituitary panel, and MRI brain
What ancillary studies need to be done before Tx?
Endocrine, audiology, vision, and neuropsychiatric studies
What is the classic appearance of craniopharyngiomas on CT/MRI?
Craniopharyngiomas are partially calcified and cystic on CT/MRI.
Is histology absolutely necessary for the Dx of craniopharyngioma?
No. A Dx can be made based on radiographic appearance and cyst fluid analysis.
What is the staging of craniopharyngioma?
There is no formal staging.
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