Endonasal endoscopic surgery
is the optimal initial treatment
for most
Craniopharyngiomas

ConditionsCraniopharyngioma

Craniopharyngiomas are benign brain tumors arising near the pituitary gland & may cause loss of pituitary hormonal function, visual loss  & headaches.

The optimal treatment for craniopharyngiomas is maximal surgical removal. Fortunately for most patients, craniopharyngiomas can be removed through a keyhole route via the nose using an endonasal endoscopic approach or an eyebrow craniotomy.  At the Pacific Pituitary Disorders Center, we have one of the world’s largest experiences treating these tumors with endoscopic and keyhole approaches as well as treating residual and recurrent craniopharyngiomas. By incorporating cutting edge technology and instrumentation with proven surgical experience, we make craniopharyngioma surgery safer, less invasive and more effective.

Most patients with a craniopharyngioma will need some form of pituitary hormone replacement and a significant minority may need radiosurgery or focused radiotherapy to prevent tumor regrowth. Our neurosurgeons work closely with our endocrinologists and radiation oncologists to provide the best possible individualized care.

Given that some craniopharyngiomas cannot be removed completely by surgery or controlled with radiation therapy, tumor genomic profiling is being increasingly used to find alternative targeted therapies for these challenging tumors. In the case of papillary craniopharyngiomas, we also profile these tumors looking specifically for the BRAF mutation that may allow treatment with a targeted drug called a BRAF inhibitor.

Experience

Endonasal Endoscopic Surgery

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Craniopharyngiomas are benign brain tumors that arise near the Pituitary Gland and pituitary stalk are typically both cystic and solid in structure.

They occur most commonly in childhood and adolescence and in later adult life, after age 50. They account for 10-15% of sellar and suprasellar tumors (tumors that occur in and above the pituitary gland) and 50-60% of sellar and suprasellar tumors in children. They are usually not discovered until they impinge upon important structures around them, and are frequently quite large (over 3 cm) when detected.

Although they are benign (not malignant) tumors, these tumors tend to become adherent to structures around the pituitary gland and stalk, including the optic nerves, optic chiasm, intracranial arteries and the brain itself.

They are thought to arise from remnants of the craniopharyngeal duct or Rathke’s pouch which are developmental structures related to the primitive gut. They are thought to be closely related to Rathke’s Cleft Cysts (RCCs).

Have you considered genomic sequencing?

We can locate which genes are the cause these tumors and find out what enables them to become resistant to treatment with radiation therapy and chemotherapy.

Learn About Your Options

Craniopharyngiomas can cause a variety of symptoms depending upon their location. 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, constipation, dry skin, nausea, low blood pressure, and depression. Pituitary stalk compression can also cause Diabetes Insipidus (DI), and increase Prolactin levels causing a milky discharge from the breast (galactohhrea). 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.

The best means of visualizing a craniopharyngioma is with a pituitary MRI. Most craniopharyngiomas are also seen on CT scan since some are partially calcified.  A complete pituitary hormonal evaluation should also be performed. Other possible diagnoses to consider with a cystic pituitary mass is a cystic pituitary adenoma or an arachnoid cyst.

  • Surgery
    The best initial treatment for a craniopharyngioma is surgical removal. The goal of surgery is to completely remove the tumor while improving vision and brain function and avoiding complications. The great majority craniopharyngiomas can be removed by either an endonasal endoscopic 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.
  • Radiosurgery (SRS) or Stereotactic Radiotherapy (SRT)
    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.
  • Hormonal Replacement Therapy
    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.