All reported extracranial craniopharyngiomas (or Rathke's pouch tumour, craniopharyngeal duct tumour) were located in the nasopharynx, sella turcica and sphenoid sinus, and presented during the first two decades of life with headaches, nasal obstruction and epistaxis (for reviews see [18, 67]). Intracranial craniopharyngiomas occur classically in an adamantinomatous and papillary subtype. The majority of the extracranial nasopharyngeal craniopharyngiomas are of the adamantinomatous subtype with cords of basaloid squamous cells and foci of squamous differentiation and horn pearls embedded in fibrous stroma. Other typical secondary changes are cysts filled with brown fluid, areas of necrosis, calcifications and cholesterol crystals. Treatment is surgical and supplemented with radiotherapy in the case of incompletely resected tumours and recurrences.

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