The following are true of Adamantinomatous craniopharyngioma except
A. Adamantinomatous variant is common in adults
B. metastatic calcification seen
C. wet keratin is diagnostic
D. palisading of squamous epithelium seen
E. arises from the vestigial remnants of rathke’sPouc
A. Adamantinomatous variant is common in adults
B. metastatic calcification seen
C. wet keratin is diagnostic
D. palisading of squamous epithelium seen
E. arises from the vestigial remnants of rathke’sPouc
Ans. A,B
The craniopharyngioma is thought to be
derived from vestigial remnants of Rathke
pouch. These slow-growing tumors account for 1% to 5% of intracranial
tumors; a small minority of these lesions arise within the sella, but most are
suprasellar [MCQ], with or without intrasellar extension. A bimodal
age distribution [MCQ]is observed, with one peak in childhood (5 to
15 years) and a second peak in adults 65 years or older. Two distinct
histologic variants are recognized: adamantinomatous
craniopharyngioma (most often observed in children) and papillary craniopharyngioma (most often
observed in adults[MCQ]). The adamantinomatous type frequently
contains radiologically demonstrable calcifications; the papillary variant
calcifies only rarely.
Adamantinomatous craniopharyngioma
consists of nests or cords of stratified squamous epithelium embedded in
a spongy “reticulum” that becomes more prominent in the internal layers. “Palisading”
[MCQ]of the squamous epithelium is frequently observed at the
periphery. Compact, lamellar keratin formation (“wet keratin”) is a
diagnostic feature of this tumor. As was mentioned above, dystrophic calcification [MCQ]
is a frequent finding. Additional features include cyst formation, fibrosis,
and chronic inflammatory reaction. The cysts of adamantinomatous
craniopharyngiomas often contain a cholesterol-rich, thick brownish-yellow
fluid. These tumors extend fingerlets of epithelium into adjacent brain, where
they elicit a brisk glial reaction.
