craniopharyngioma

Etiology

• slow growing, encapsulated squamous cell tumor derived from remnants of Rathke's pouch (craniopharyngeal duct).

Epidemiology

• most common tumor in the pituitary region in childhood
• may occur at any age

Pathology

• 2/3 of the tumors are suprasellar
• 1/3 originate or extend into the sella turcica
• most tumors are cystic
  • brown cholesterol containing fluid ('machinery oil')
• some are solid or mixed
• propensity to calcify
• presumably derived from remnants of embryonic pituitary

Microscopic pathology

• adamantinomatous epithelium
• loose arrangement of squamous cells
• nodules of keratin
• fibrosis
• cholesterol deposits

Clinical manifestations

• obstructive hydrocephalus
• hypothalamic syndrome (injury)
  • diabetes insipidus
  • hyperprolactinemia
  • increased appetite, thirst, weight gain, morbid obesity
  • temperature dysregulation
  • sleep disorder
• optic chiasmal defects
• panhypopituitarism

Laboratory

• serum sodium: hypontremia (postoperative SIADH)
  • long-term central diabetes insipidus
• also see panhypopituitarism

Radiology

• plain radiographs:
  • calcification in or around the sella turcica
• computed tomography (CT)
  • solid, cystic or mixed solid-cystic mass
  • calcification
• magnetic resonance imaging (MRI)
  • solid, cystic or mixed solid-cystic mass
  • low attenuation values

* image

Differential diagnosis

• non-functioning pituitary adenoma (macroadenoma)
  • generally not cystic, located in sella turcica not suprasellar region

Management

• surgical excision is feasible only for small tumors
• surgical decompression of larger tumors
• ventriculoperitoneal shunt for obstructive hydrocephalus
• post-operative radiation therapy
• management of endocrine dysfunction

More general terms

• neuroectodermal neoplasm
• brain neoplasm (intracranial neoplasm)

Patient information

craniopharyngioma patient information