Craniopharyngioma history and symptoms

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Marjan Khan M.B.B.S.[2]

Craniopharyngiomas are slow growing and symptoms often are present for a year or more before the diagnosis is established. A wide range of symptoms may be present, depending upon the precise location of the tumor and its relationship to adjacent normal structures. Most common symptoms of craniopharyngioma include headache, nausea, vomiting, ataxia, polyuria, polydipsia, stunted growth, decreased libido, amenorrhea, weight gain, myxedema, vision loss, behavioral and learning problems.

Craniopharyngioma causes symptoms by:^[1]

• Increasing pressure on the brain usually from obstructive hydrocephalus: ^[2]
  • Headache
  • Nausea
  • Vomiting (especially in the morning)
  • Ataxia
• Disrupting hormone production by the pituitary gland:
  • Polyuria
  • Polydipsia
  • Stunted growth, leading to short stature
  • Delayed puberty in children
  • Decreased libido
  • Amenorrhea
  • Weight gain
  • Myxedema
• Pressure or damage to the optic nerve: ^[2]
  • Vision loss (bitemporal hemianopsia) ^[3]
  • 20% of children and 80% of adults with craniopharyngioma have visual defects.
  • These defects are often permanent, and may get worse after surgical removal of the tumor. ^[3]
• Behavioral and learning problems may be present, due to frontal and temporal extension

• Visual symptoms are frequent, and deficits on formal ophthalmologic assessment are present in the majority of patients. ^[2]
• Symptoms can be a direct result of pressure on the optic chiasm.
• The specific deficit depends upon the growth pattern of the tumor.

• Direct damage to or compression of normal structures can lead to a range of endocrine abnormalities.
• Most frequently observed complications include deficiencies of growth hormone, gonadotropin, thyroid stimulating hormone, and adrenocorticotropic hormone.
• Diabetes insipidus is frequent when the pituitary stalk is involved. ^[3]
• Growth failure, which can be caused by either hypothyroidism or growth hormone deficiency.
• Sexual dysfunction is the most common endocrine manifestation in adults.
• 90% of men complain of erectile dysfunction, while most women have amenorrhea.^[4]

• Headaches are present in approximately 50 percent of patients at the time of diagnosis. ^[5]
• Headaches may result from traction on pain-sensitive structures by the tumor itself, obstructive hydrocephalus from tumor compression of the third ventricle, or meningeal irritation by escaped cyst contents.^[6]

• Craniopharyngiomas can also cause other generalized symptoms, such as depression, independent of any hormone deficiency.^[2]
• The presumed cause is extension of tumor into the frontal lobes, striatal and thalamic areas, or limbic system.
• Nausea, vomiting, and lethargy can accompany pressure-related headaches in craniopharyngioma.^[3]

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