Makindo Medical Notes

🧠 Craniopharyngioma is a benign embryological tumour near the pituitary stalk. Although histologically non-cancerous ✅, its proximity to critical structures can cause significant morbidity. Early recognition is key to preserving vision 👀 and endocrine function 🧬.

📖 About Craniopharyngioma

Originating from Rathke’s 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 👧 and middle-aged adults 👩‍🦳.

• Embryological remnant of Rathke’s pouch 🧬
• Sellar/suprasellar location 🪑
• Bimodal age peak: children & middle-aged adults 📆

⚙️ Aetiology

• Failure of Rathke’s pouch remnants to regress.
• Often cystic, calcified 🪨 (classic feature on CT).
• Adhere to adjacent structures → difficult surgical resection ⚠️

🔎 Clinical Presentation

• 👀 Visual impairment: Bitemporal hemianopia from optic chiasm compression.
• 🧬 Hormonal issues: Growth delay, hypopituitarism, infertility.
• 🍽️ Hypothalamic symptoms: Hyperphagia → obesity, temperature & thirst dysregulation.
• 💧 Cranial DI: Polyuria + polydipsia.
• 🤕 Raised ICP: Headaches, nausea, papilloedema.

🧾 Differential Diagnosis

• 🎗️ Pituitary adenoma
• 🧩 Meningioma
• 👁️ Optic glioma
• 🧠 Glioblastoma
• 🌊 Choroid plexus papilloma

🧪 Investigations

• 🩸 Pituitary hormone profile (cortisol, GH, TFTs, PRL, sex hormones).
• 🖼️ MRI: Preferred; shows cystic, suprasellar lesion.
• 🪨 CT: Detects calcifications.
• 👁️ Visual fields: Bitemporal hemianopia.
• 💧 Osmolality testing: For cranial DI.

🛠️ Management

• 🔪 Surgery: Mainstay (transsphenoidal if sellar, craniotomy if suprasellar).
• 🚰 CSF shunt: If hydrocephalus present.
• ☢️ Radiotherapy: If incomplete resection or recurrence.
• 💊 Hormone replacement: Lifelong if pituitary damage (hydrocortisone, thyroxine, sex hormones, desmopressin).
• 📅 Follow-up: Imaging + endocrine monitoring for recurrence or delayed deficiency.

📚 Case Example

👦 Case: A 12-year-old boy presents with progressive visual difficulty in school 👓 and worsening headaches 🤕. Exam: Bitemporal hemianopia. Height <3rd percentile, delayed puberty. 🪨 CT: Calcified suprasellar mass. 🖼️ MRI: Cystic + solid tumour compressing optic chiasm. 🧬 Hormones: Low GH + TSH, cortisol borderline. ✅ Diagnosis: Craniopharyngioma. 🛠️ Management: Neurosurgical excision + endocrine replacement.

🌟 Prognosis

• ✅ Good survival (benign histology).
• ⚠️ High morbidity: endocrine dysfunction (50–90% need lifelong replacement).
• 👀 Vision may improve if treated early, but field defects often persist.
• 🔄 Recurrence common → lifelong monitoring required.