Description

• Benign epithelioid tumor, usually suprasellar, partially calcified, solid, or mixed solid–cystic that arises from remnants of the craniopharyngeal duct or Rathke pouch
• Histologic subtypes
  • Adamantinomatous craniopharyngioma; more common in children
  • Papillary craniopharyngioma; more common in adults

Epidemiology

Bimodal age distribution

• Children between 5 and 14 years
• Older adults between 50 and 74 years

Incidence
0.5 to 2.0 new cases per million population per year

Prevalence

• ~2/100,000 persons
• More common in countries other than the United States (e.g., Africa and Japan)

Etiology and Pathophysiology

• Eye: Pressure on the optic chiasm results in visual changes.
• Endocrine: Pressure on hypothalamus/pituitary results in deficiencies of growth hormone (GH), gonadotropins (Gn), thyroid-stimulating hormone (TSH), antidiuretic hormone (ADH), and adrenocorticotropic hormone (ACTH).
  • GH deficiency: growth failure
  • Gn deficiency: erectile dysfunction (ED), amenorrhea, delayed puberty
  • TSH deficiency: hypothyroidism
  • ADH deficiency: diabetes insipidus (DI)
  • ACTH deficiency: hypoadrenalism
• Pain: headaches (from increased intracranial pressure), hydrocephalus (tumor compression of the 3rd ventricle), or meningeal irritation by escaped cyst contents
• GI: nausea, vomiting
• Cardiovascular: hypertension (HTN) and/or orthostatic hypotension
• Not well understood. Theories include the following:
  • Adamantinomatous type may result from altered embryogenesis, in which remnant squamous cells proliferate from their origin in the sellar region into the median eminence while Rathke pouch is transforming into the anterior pituitary.
  • Papillary type may result from metaplasia of cells in the pars tuberalis of the adenohypophysis.

Risk Factors

Unknown

Commonly Associated Conditions

• DI: common when pituitary stalk is involved
• Sexual dysfunction (common)
• Depression
• Sleep apnea
• Turner syndrome (rare)