Suprasellar and pineal tumours: craniopharyngioma
- Lesion
Some tumours near the pituitary come from leftover embryonic tissue, not from hormone cells. They press on the hypothalamus and the optic nerves.
What it is
The suprasellar region hosts tumours that cause hypopituitarism, diabetes insipidus and visual loss by pressure. Craniopharyngioma, derived from remnants of Rathke pouch, is the main one and has two forms with different genetics and ages. Hypothalamic and optic pathway gliomas also arise here, especially in children. The pineal gland gives rise to rare parenchymal tumours whose behaviour follows their differentiation; most pineal masses are germ-cell tumours.
Structure
- Adamantinomatous: nests of squamous epithelium with peripheral palisading, loose stellate reticulum, wet keratin nodules, calcification, cholesterol-rich cyst fluid and a gliotic brain interface.
- Papillary: well-differentiated non-keratinising squamous epithelium around fibrovascular cores.
- Pineocytoma: sheets of small mature cells forming large pineocytomatous rosettes.
- Pineoblastoma: dense sheets of primitive small cells with high mitotic activity, like medulloblastoma.
Two craniopharyngiomas compared
| Feature | Adamantinomatous | Papillary |
|---|---|---|
| Age | Children (and a second adult peak) | Adults |
| Driver | CTNNB1, nuclear beta-catenin | BRAF V600E |
| Gross | Cystic, calcified, machinery-oil fluid | Mostly solid |
| Keratin | Wet keratin nodules | None |
| Recurrence | Common, sticks to hypothalamus | Less common |
Clinical notes
- Children present with growth failure, visual loss, headache and diabetes insipidus.
- Surgery is hard because the tumour adheres to the hypothalamus; obesity and hyperphagia can follow.
- BRAF inhibitors are an option for papillary tumours.
Test yourself
Think of the answer, then tap the question to see it.
Which mutation defines adamantinomatous craniopharyngioma?
Activating CTNNB1 mutation, with nuclear accumulation of beta-catenin.
What is the pathologic hallmark of adamantinomatous craniopharyngioma on H&E?
Squamous nests with palisaded periphery, stellate reticulum, wet keratin and calcification.
Why does a pineal tumour cause upward gaze palsy?
It compresses the dorsal midbrain (tectum), producing Parinaud syndrome.
Elsewhere in DurgiPedia
Connections
Educational content on the mechanisms of disease; not a diagnostic or treatment guide and no substitute for a health professional's advice. No doses are given.
Sources: Childhood Craniopharyngioma Treatment (PDQ®)–Health Professional Version (National Cancer Institute, 2025).
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Representative model, for educational purposes only.