Brain, Papillary craniopharyngioma [LMP80549]

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Diagnostic Modality: 
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Disease Category: 
Neoplasia
Diagnosis: 
Papillary craniopharyngioma
Species: 
Human
Organ System: 
Central Nervous System
Gender: 
Male
Age: 
54 years
Diagnosis Code: 
ICD-10: C71 | ICD-O: 9352/1
Slide Contributor Information
Gao, A., Munoz, D.

Keywords

sella, sellar tumour, BRAF V600E

Clinical History

54-year-old man presents with visual changes. Neuroimaging shows a suprasellar mass.

Clinical Discussion

Papillary craniopharyngioma is a variant of craniopharyngioma, a tumour of the sellar/suprasellar region classified as WHO grade I. Unlike the adamantinomatous variant, papillary craniopharyngioma typically occurs in adults instead of children. Microscopically, it is composed of well-differentiated sheets of non-keratinizing squamous epithelium with crude papillae formed by tumour dehiscence around fibrovascular cores. They lack the wet keratin, conspicuous basal palisading, and loose stellate reticulum seen in the adamantinomatous variant. Papillary craniopharyngiomas harbour the BRAF V600E mutation (see related content), while adamantinomatous craniopharyngiomas have beta-catenin mutations (and nuclear beta-catenin expression). Prognosis is good with gross total resection.

Related Content

BRAF V600E