Craniopharyngioma

• Headaches
• Visual disturbances
• Hormonal imbalances
• Growth abnormalities
• Cognitive or behavioral changes
• Weight changes
• Increased intracranial pressure.

If the tumor compresses the pituitary stalk or gland, the tumor can cause partial or complete pituitary hormone deficiency which may lead to:

• Growth failure
• Delayed puberty
• Loss of normal menstrual function or sexual desire
• Increased sensitivity to cold
• Fatigue
• Diabetes insipidus – leading to increased prolactin hormone levels causing a milky discharge from the breast (galactorrhea)
• Constipation
• Dry skin
• Nausea
• Low blood pressure
• Depression

If the tumor compresses the optic chiasm or nerves, then visual loss can result.

Involvement of the hypothalamus, an area at the base of the brain, may result in obesity, increased drowsiness and temperature regulation abnormalities.

Other symptoms especially with larger tumors may include personality changes, headache, confusion, and vomiting. Large craniopharyngiomas which extend upward toward the fluid filled ventricles of the brain can cause hydrocephalus.

The best initial treatment for a craniopharyngioma is surgical removal. The goal of surgery is maximal safe tumor removal while improving vision and brain function and avoiding complications. If a patient has multiple pituitary hormonal deficiencies before surgery, it is reasonable to try for a more complete tumor removal. However, if pituitary function is largely normal, a more conservative surgical removal approach may be recommended in an effort to preserve gland function. The great majority of craniopharyngiomas can be removed by either an endoscopic endonasal approach (through the nose) or a supra-orbital eyebrow craniotomy. Because of their tendency to be adherent to the optic chiasm, other nerves and important blood vessels, a total removal is possible in only 50 – 60% of patients.

With incomplete removal, stereotactic radiotherapy (SRT) or stereotactic radiosurgery (SRS), are typically used to prevent further tumor growth. Additionally, because of the tendency for craniopharyngiomas to recur, repeat MRI should be obtained at least every six months for the first 5 years after surgery or radiation and then at least annually thereafter.

Many patients with a craniopharyngioma will develop pituitary hormonal deficits because of the tumor itself, surgery or as a result of radiotherapy. Such patients require hormone replacement therapy which may include thyroid, cortisol, testosterone (men), estrogen (women) and/or DDAVP for diabetes insipidus. Because hormonal deficiencies can develop years after radiotherapy, patients should have periodic hormonal evaluations throughout their lifetimes. Regular follow-up with an endocrinologist is recommended for all patients with a craniopharyngioma.

With expert treatment, most people with craniopharyngioma can achieve good long-term tumor control and quality of life. However, because the tumor grows near critical brain structures and the pituitary gland, treatment may lead to lifelong hormonal changes and the need for ongoing medical care. Regular follow-up with imaging and hormone testing is essential, as craniopharyngiomas can recur even years after initial treatment.

Craniopharyngiomas are benign (non-cancerous) tumors, and the long-term survival rate is generally high—over 90% at 10 years with appropriate treatment. Survival depends more on managing complications, recurrences, and hormonal deficiencies than on the tumor itself.

Symptoms in adults vary depending on the tumor’s size and location, and may include:

• Persistent headaches
• Vision problems (blurry vision, loss of part of the visual field)
• Hormonal changes (fatigue, weight changes, loss of libido, menstrual changes in women)
• Increased thirst and urination (diabetes insipidus)
• Cognitive or personality changes
• Nausea, vomiting, or balance issues if pressure inside the skull increases

Long-term effects are often related to the tumor’s location and the treatments required, and may include:

• Lifelong need for pituitary hormone replacement
• Vision loss (partial or complete)
• Memory or concentration difficulties
• Fatigue
• Weight gain or difficulty regulating body temperature (if the hypothalamus is affected)
• Risk of tumor recurrence, requiring ongoing monitoring

Treatment usually involves a multidisciplinary approach and may include:

• Surgery (often endoscopic endonasal or eyebrow craniotomy) to remove as much of the tumor as safely possible
• Stereotactic radiosurgery (SRS) or stereotactic radiotherapy (SRT) for residual or recurrent tumor
• Hormone replacement therapy to manage pituitary deficiencies
• Targeted drug therapy in certain tumor types, such as BRAF inhibitors for papillary craniopharyngiomas

Most patients live a normal life span after successful treatment, though they may require lifelong medical follow-up and hormone replacement. Life expectancy is influenced more by treatment complications, hormonal imbalances, or other health conditions than by the tumor itself.

Death from craniopharyngioma is rare, but when it occurs, it is usually due to:

• Severe, untreated hormonal deficiencies (such as adrenal insufficiency)
• Complications from surgery or radiation
• Progressive tumor growth affecting critical brain structures
• Rarely, severe hypothalamic injury leading to life-threatening metabolic disturbances

While both tumors occur in the sellar and suprasellar region, there are key differences:

• Origin: Pituitary adenomas arise from the pituitary gland itself, while craniopharyngiomas develop from embryonic remnants near the gland.
• Imaging: On MRI, craniopharyngiomas often appear as cystic (fluid-filled) masses with calcifications, whereas pituitary adenomas are usually solid without calcifications.
• Symptoms: Both can cause hormonal changes and vision problems, but craniopharyngiomas more often cause severe hypothalamic symptoms, such as obesity or temperature regulation issues.
• Treatment approach: Both require specialized neurosurgical care, but craniopharyngiomas more often need a combined approach of surgery, radiation, and lifelong hormone replacement.