Craniopharyngioma

What is a Craniopharyngioma?

Craniopharyngiomas are rare, benign (WHO Grade I) tumors that arise from remnants of Rathke's pouch along the craniopharyngeal duct. They are typically suprasellar in location and can cause symptoms through mass effect on the pituitary, hypothalamus, and optic pathways.

Types:

  • Adamantinomatous: More common in children; often calcified with cystic components.
  • Papillary: More common in adults; solid tumors, less calcification.

Clinical Features:

  • Visual Disturbances: Bitemporal hemianopia due to optic chiasm compression.
  • Endocrine Deficiencies: Growth hormone deficiency (most common), hypogonadism, hypothyroidism, adrenal insufficiency.
  • Diabetes Insipidus: Due to compression of the pituitary stalk.
  • Headache and Hydrocephalus: Large tumors can obstruct CSF flow.

Treatment:

  • Surgery: Gross total resection is curative but challenging due to adherence to surrounding structures.
  • Radiation: For residual or recurrent disease (stereotactic radiosurgery or fractionated RT).
  • Hormone Replacement: Lifelong endocrine follow-up is essential.

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