Endocrinology, Diabetes, and Metabolism· Topic 4.8 Hypothalamic-pituitary axis and water homeostasis· 4.8.2

Craniopharyngioma

Craniopharyngioma is a slow-growing, encapsulated, often calcified squamous epithelial tumor arising from Rathke pouch remnants along the pituitary stalk, with a bimodal peak at ages 5 to 15 and 45 to 60. It is the most common extrasellar cause of hypothalamic-pituitary failure and presents with headache, bitemporal hemianopsia, hypopituitarism, central diabetes insipidus, hypothalamic obesity, or obstructive hydrocephalus; treatment is surgical resection or decompression with postoperative radiotherapy for residual tumor, plus lifelong replacement of every deficient axis, starting with hydrocortisone.

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What this article covers

  • Overview

    Definition: craniopharyngioma is a slow-growing, encapsulated squamous cell tumor originating from epithelial remnants of Rathke pouch within the pituitary stalk; it is histologically benign but associated with a high recurrence rate.

  • Epidemiology

    Most common tumor of the pituitary region in childhood and the most common supratentorial brain tumor in children, an exception to the infratentorial predominance of pediatric brain tumors.

  • Risk factors

    Age in either peak (5 to 15 years or 45 to 60 years) is the main association.

  • Pathophysiology

    Rathke pouch is an evagination of oral (surface) ectoderm that forms the anterior pituitary; epithelial remnants left along the pituitary stalk can give rise to craniopharyngioma.

  • Clinical features

    Mass effect is the presenting hallmark, after years of subtle symptoms: progressive headache and visual field defects, classically bitemporal hemianopsia from optic chiasm compression; papilledema…

  • Differential diagnosis

    Nonfunctioning pituitary adenoma: the most common sellar mass, solid and arising within the sella; calcification is common in craniopharyngioma and distinguishes it from adenoma.

  • Diagnosis

    The diagnosis is established by imaging and confirmed histologically: a sellar or suprasellar mass with cystic and solid components on MRI plus calcification (best seen on CT) and low attenuation…

  • Management

    First, if there is obstructive hydrocephalus with raised intracranial pressure, obtain emergent neurosurgical decompression; a ventriculoperitoneal shunt treats the obstructive hydrocephalus.

  • Complications

    Adrenal crisis from unrecognized central adrenal insufficiency, particularly perioperatively or when levothyroxine is started before hydrocortisone.

  • Prognosis

    Prognosis is variable; many survivors have lasting endocrine or visual deficits from the tumor's location.

  • Prevention

    Screen every patient before surgery with a morning cortisol: below 3 mcg/dL treat as deficient, 3 to 15 mcg/dL perform an ACTH stimulation test, and above 15 mcg/dL deficiency is excluded.

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