Fact-sheet: Craniopharyngioma
Updated on 03/26/2020 at 1:59 PM
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Definition
Benign tumor arising from undifferentiated ectoblastic remnants of the Rathke cleft.
2 peaks of incidence:
- young children (5-15 years)
- adults (50-70 years)
2 main histopathologic forms:
- Adamantinomatous form
- Papillary and squamous form
Locations:
- 70% are intra- and suprasellar
- 20% are suprasellar only
- 10% are intrasellar only
- Exceptionally: intraventricular, sphenoidal, or nasopharyngeal
Clinical features
Clinical presentation depends on tumor compression:
- Endocrine disorders (delayed puberty, growth disturbances, diabetes insipidus)
- Visual disturbances
- Clinical picture of intracranial hypertension
CT
Heterogeneous mass with enhancing solid component.
Cystic portion, fatty or spontaneously hyperdense.
Calcifications (mainly present in adamantinomatous forms)
MRI
Reference examination for preoperative locoregional workup and postoperative follow-up.
3 components: solid, cystic, and calcific
- solid portion enhances after contrast administration, hypo- or isointense on T1.
- cystic area: generally hypointense on T1 and hyperintense on T2. Sometimes hyperintense on T1 due to the presence of methemoglobin, cholesterol, or proteins.
- calcifications: undetected if small, otherwise hypointense. Better seen on gradient-echo T2*.
- perilesional parenchymal edema.
Other modalities:
Diffusion: cystic portion iso- or hyperintense relative to brain parenchyma.
Spectroscopy: lipid peak
Management
Surgical resection.
Radiotherapy.
High risk of recurrence ++ therefore MRI follow-up
Classification
WHO grade I
Differential diagnosis
Meningioma
Chiasmatic glioma
Dermoid cyst
Rathke cleft cyst
Astrocytoma
Pituitary adenoma
Germinoma