![• Craniopharyngiomas usually arise along the pituitary stalk in
the suprasellar region adjacent to the optic chiasm. A small
percentage arises within the sella [8,9], and even fewer along
the optic system or within the third ventricle [](https://image.slidesharecdn.com/craniopharyngioma-221127050859-83a53adf/85/CRANIOPHARYNGIOMA-pptx-1-320.jpg)
![Adamantinomatous craniopharyngiomas are characterized by activation of the
Wnt signaling pathway, and almost all harbor activating mutations
in CTNNB1, the gene encoding β-catenin [13-15]. By contrast, papillary
craniopharyngiomas commonly harbor mutations in the BRAF oncogene
Headache – Moderate to severe daily headaches are present in
approximately 50 percent of patients at the time of diagnosis [20]. These
may result from traction on pain-sensitive structures by the tumor itself,
obstructive hydrocephalus from tumor compression of the third
ventricle, or meningeal irritation by escaped cyst contents.](data:image/gif;base64,R0lGODlhAQABAIAAAAAAAP///yH5BAEAAAAALAAAAAABAAEAAAIBRAA7)
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CRANIOPHARYNGIOMA.pptx
- 1. • Craniopharyngiomas usually arise along the pituitary stalk in the suprasellar region adjacent to the optic chiasm. A small percentage arises within the sella [8,9], and even fewer along the optic system or within the third ventricle [
- 2. Adamantinomatous craniopharyngiomas are characterized by activation of the Wnt signaling pathway, and almost all harbor activating mutations in CTNNB1, the gene encoding β-catenin [13-15]. By contrast, papillary craniopharyngiomas commonly harbor mutations in the BRAF oncogene Headache – Moderate to severe daily headaches are present in approximately 50 percent of patients at the time of diagnosis [20]. These may result from traction on pain-sensitive structures by the tumor itself, obstructive hydrocephalus from tumor compression of the third ventricle, or meningeal irritation by escaped cyst contents.
- 3. • Calcification in the suprasellar region is seen in 60 to 80 percent of patients with craniopharyngioma, and one or more cysts are present in approximately 75 percent. A cystic calcified parasellar lesion is very likely to be a craniopharyngioma (image 1 and image 2). CT or plain skull radiographs can help distinguish adamantinomatous craniopharyngiomas from noncalcified suprasellar lesions. Papillary craniopharyngiomas frequently lack calcification
- 4. • • General features ○ Multilobulated, often large (> 5 cm) ○ Occasionally giant, multicompartmental • CT ○ Cystic (90%), Ca++ (90%), enhancing (90%) • MR: Signal varies with cyst contents ○ Cysts variably hyperintense on T1WI and T2WI ○ Solid portions enhance heterogeneously; cyst walls enhance strongly ○ Cyst contents show broad lipid peak (0.9-1.5 ppm) on MR spectroscopy
- 5. • • Location ○ Surgical division of CPs into 3 groups – Sellar – Prechiasmatic – Retrochiasmatic ○ Imaging locations of CPs (adamantinomatous type) – Suprasellar (75%) – Suprasellar + intrasellar component (21%) – Entirely intrasellar (4%) – Often extends into multiple cranial fossae: Anterior (30%), middle (23%), posterior, &/or retroclival (20%