Craniopharyngioma
Epidemiology
and Etiology:
- Bimodal
age distribution
- Differentiation
similar to vestigial remnants of Rathke’s pouch
Common
sites:
- Suprasellar (most)
- intrasellar
Gross
features:
- 3-4cm
average
- Often
cystic, multiloculated
- Calcified
(adamantinomatous craniopharyngioma)
-
Histologic
features:
- Adamantinomatous craniopharyngioma:
- Nests
and cords of stratified squamous epithelium
- Layer
of columnar cells peripherally resting on a basement membrane
- Calcifications
frequently
- Embedded
in a spongy reticulum
- Extending
fingerlets into surrounding brain
- Fibrosis
often
- Chronic
inflammation often
-
- Papillary
craniopharyngioma:
- Solid
sheets and papillae lined by well-differentiated squamous
epithelium
- Lacking
features of adamantinomatous craniopharyngioma:
- Usually
lacking keratin, calcifications, and cysts
- No
peripheral pallisading in solid areas
- Lack
of spongy reticulum
Immunophenotype:
Marker:
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Sensitivity:
|
Specificity:
|
|
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Molecular
features:
Other
features:
- Commonly
causes hyper / hypopituitarism or visual
disturbances
- Excellent
recurrence-free and overall survival
References:
- Robbins
& Cotran Pathologic Basis of Disease (2005)