"MRI "
"T1: iso- to hyperintense to grey matter 11 (due to high protein content "motor oil cysts")"
"T2: variable but ~80% are mostly or partly T2 hyperintense"
"T1 C+ (Gd): vivid enhancement"
"T2: variable or mixed"
"MR angiography: may show displacement of the A1 segment of the anterior cerebral artery (ACA)"
"MR spectroscopy: cyst contents may show a broad lipid spectrum, with an otherwise flat baseline 8"
"Treatment requires a multidisciplinary approach. For tumours without hypothalamic involvement, gross total resection remains a goal when safely achievable. For tumours with hypothalamic involvement, which unfortunately is the majority of adamantinomatous craniopharyngiomas, current evidence-based guidelines recommend hypothalamus-sparing limited surgery (accepting residual tumour) followed by adjuvant radiotherapy to the tumour remnant, rather than aggressive radical resection16. This approach reduces the risk of hypothalamic obesity, panhypopituitarism, and neurocognitive sequelae."
"Adamantinomatous craniopharyngiomas are far more common than papillary craniopharyngiomas and are identified in all age groups, but predominantly in children with a second, less pronounced, peak in later life (40-69 years of age) 9,15. No sex predilection has been identified 9. In the USA incidence is highest in Black patients and lowest in American Indian/Alaska Natives 15."
"Increasing molecular evidence has demonstrated that they have distinct and mutually exclusive mutations of CTNNB1 (adamantinomatous) and BRAF (papillary) 1,2,4. As such, they are now considered separate entities and are discussed separately."
"Adamantinomatous craniopharyngiomas have β-catenin gene (CTNNB1) mutations that prevent degradation of β-catenin protein 2,4. Only rarely do they also harbour BRAF V600E mutations 13 which are, instead, characteristic of papillary craniopharyngiomas 14."
"Rathke cleft cyst"
Expected headings
"MRI "