Craniopharyngioma
๐ง Craniopharyngioma is a benign embryological tumour near the pituitary stalk.
Although histologically non-cancerous โ
, its proximity to critical structures can cause significant morbidity.
Early recognition is key to preserving vision ๐ and endocrine function ๐งฌ.
๐ About Craniopharyngioma
Originating from Rathkeโs pouch remnants, craniopharyngiomas typically sit in the sellar/suprasellar region.
They often adhere to the optic chiasm, pituitary, and hypothalamus, making surgery challenging.
Commonest in children ๐ง and middle-aged adults ๐ฉโ๐ฆณ.
- Embryological remnant of Rathkeโs pouch ๐งฌ
- Sellar/suprasellar location ๐ช
- Bimodal age peak: children & middle-aged adults ๐
โ๏ธ Aetiology
- Failure of Rathkeโs pouch remnants to regress.
- Often cystic, calcified ๐ชจ (classic feature on CT).
- Adhere to adjacent structures โ difficult surgical resection โ ๏ธ
๐ Clinical Presentation
- ๐ Visual impairment: Bitemporal hemianopia from optic chiasm compression.
- ๐งฌ Hormonal issues: Growth delay, hypopituitarism, infertility.
- ๐ฝ๏ธ Hypothalamic symptoms: Hyperphagia โ obesity, temperature & thirst dysregulation.
- ๐ง Cranial DI: Polyuria + polydipsia.
- ๐ค Raised ICP: Headaches, nausea, papilloedema.
๐ก Clinical Pearl:
Child with short stature, delayed puberty + bitemporal hemianopia + calcified suprasellar mass โ think craniopharyngioma.
๐งพ Differential Diagnosis
- ๐๏ธ Pituitary adenoma
- ๐งฉ Meningioma
- ๐๏ธ Optic glioma
- ๐ง Glioblastoma
- ๐ Choroid plexus papilloma
๐งช Investigations
- ๐ฉธ Pituitary hormone profile (cortisol, GH, TFTs, PRL, sex hormones).
- ๐ผ๏ธ MRI: Preferred; shows cystic, suprasellar lesion.
- ๐ชจ CT: Detects calcifications.
- ๐๏ธ Visual fields: Bitemporal hemianopia.
- ๐ง Osmolality testing: For cranial DI.
๐ ๏ธ Management
- ๐ช Surgery: Mainstay (transsphenoidal if sellar, craniotomy if suprasellar).
- ๐ฐ CSF shunt: If hydrocephalus present.
- โข๏ธ Radiotherapy: If incomplete resection or recurrence.
- ๐ Hormone replacement: Lifelong if pituitary damage (hydrocortisone, thyroxine, sex hormones, desmopressin).
- ๐
Follow-up: Imaging + endocrine monitoring for recurrence or delayed deficiency.
๐ Case Example
๐ฆ Case: A 12-year-old boy presents with progressive visual difficulty in school ๐ and worsening headaches ๐ค.
Exam: Bitemporal hemianopia. Height <3rd percentile, delayed puberty.
๐ชจ CT: Calcified suprasellar mass. ๐ผ๏ธ MRI: Cystic + solid tumour compressing optic chiasm.
๐งฌ Hormones: Low GH + TSH, cortisol borderline.
โ
Diagnosis: Craniopharyngioma.
๐ ๏ธ Management: Neurosurgical excision + endocrine replacement.
๐ Prognosis
- โ
Good survival (benign histology).
- โ ๏ธ High morbidity: endocrine dysfunction (50โ90% need lifelong replacement).
- ๐ Vision may improve if treated early, but field defects often persist.
- ๐ Recurrence common โ lifelong monitoring required.