Overview Flashcards
What is the origin of craniopharyngioma?
Epithelial tumor derived from Rathke pouch, the embryonic precursor to the ant pituitary
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 behave aggressively with frequent LRs and high risk of morbidityd/t the location of Dz.
Appx how many cases of craniopharyngioma occur annually in the United States?
∼300–350 cases/yr of craniopharyngioma in the United States, accounting for 1%–3% of all pediatric brain tumors.
At what ages does craniopharyngioma occur?
Commonly occur b/t ages 5–10 yrs. There is a bimodal distribution (5–15 yrs and 45–60 yrs); one-third of cases occur in pts aged 0–14 yrs.
What are the 2 histologic subtypes of craniopharyngioma?
Adamantinomatous and papillary; thought of as WHO grade 1 tumors.
Which subtype is characterized by a solid and cystic pattern?
Adamantinomatous craniopharyngioma has a solid and cystic pattern. A recent study suggests this histology has more frequent LR. (Pekmezci M et al., Neurosurgery 2010)
Historically, how has the cyst fluid consistency been described?
“Crankcase (machine) oil”–like (very proteinaceous fluid with cholesterol crystals)
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 (bitemporal hemianopsia)
Endocrinopathies (TSH, GH, LH/FSH).
What is the most common hormone deficiency at presentation?
At presentation, GH is the most common hormone deficiency.
Do craniopharyngioma tumors respond rapidly or slowly to RT?
Craniopharyngioma tumors respond slowly to RT.
What is the main histology for craniopharyngioma in children, and what are its characteristics? How does this differ from the histology seen in adults?
The major histology seen in childhood craniopharyngioma is adamantinomatous. These tumors are typically calcified, comprised of solid components, and large complex cysts filled with lipid-laden fluid described as “crankcase oil.” Squamous cell histology is seen almost exclusively in adults. These tumors tend to be solid, without cystic components.
What endocrine abnormalities are most commonly seen at time of diagnosis of craniopharyngiomas?
At diagnosis, 50% to 90% of pediatric patients will present with endocrine abnormalities, with growth hormone deficiency being the most common, though DI can be seen in 10% to 20% of cases as well. Sexual dysfunction is the most frequently seen endocrine abnormality in adults.
