Surgery for Craniopharyngiomas Clinical Presentation

Updated: May 03, 2022
  • Author: Lawrence S Chin, MD, FACS, FAANS; Chief Editor: Brian H Kopell, MD  more...
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Presentation

History and Physical Examination

The onset of symptoms is generally insidious, with a delay of approximately 1-2 years between initial symptoms and diagnosis. The clinical presentation can include a wide range of symptoms, which depend on the location of the tumor and involvement of adjacent structures. Headache is the most common presenting symptom, followed by endocrine deficiencies and visual disturbances.

Headache is usually due to either the tumor’s mass effect or hydrocephalus (from obstruction of the foramen of Monro, third ventricle, or aqueduct of Sylvius), which occurs in 15-30% of patients. This obstructive hydrocephalus may, on rare occasions, necessitate emergency neurosurgical management.

Endocrine disturbances are related to direct compression from the tumor. Endocrine deficiency most commonly involves growth hormone (75%), followed by gonadotropin (40%), thyroid-stimulating hormone (25%), and corticotropin (25%). [8]  Growth failure may be noted in as many as 93% of children with craniopharyngioma and is related to growth-hormone deficiency, hypothyroidism, or both. Adults have more varied presentation and may develop sexual or menstrual dysfunction, with 88% of men experiencing decreased sex drive and 82% of women experiencing amenorrhea. Other endocrine dysfunction may lead to precocious puberty and obesity.

Large tumors in adults can cause psychiatric symptoms, memory loss, apathy, incontinence, depression, and hypersomnia. Long-standing cognitive deficits and profound memory loss have been reported and suggest a worse prognosis. Visual deficits are caused by compression of the optic chiasm from suprasellar tumor growth. Classically, the tumor presents as a bitemporal hemianopsia, but it may also manifest as homonymous hemianopsia, scotoma, or optic atrophy with papilledema.

Other presenting symptoms can include chemical meningitis from rupture of cyst contents into the subarachnoid space and resulting in headaches, stiff neck, and seizures.