Craniopharyngioma

Craniopharyngioma begins near the brain's pituitary gland, which secretes hormones that control many body functions. As the craniopharyngioma grows slowly, it can affect the function of the pituitary gland and other nearby structures in the brain.

Craniopharyngioma can occur at any age, but is most common in children and older adults. Symptoms include gradual changes in vision, fatigue, excessive urination, and headaches. Children with craniopharyngioma may grow slowly and be smaller than expected.

Diagnosis

Tests and procedures used to diagnose craniopharyngioma include:

Physical examination. Diagnosing a craniopharyngioma usually begins with a medical history review and a neurological exam by your doctor. During this procedure, vision, hearing, balance, coordination, reflexes, growth and development are tested.

blood tests. Blood tests can reveal changes in hormone levels that indicate a tumor is affecting your pituitary gland.

Imaging tests. Tests to create images of your brain may include X-rays, magnetic resonance imaging (MRI), and computed tomography (CT).

Treatment

Craniopharyngioma treatment options include:

Operation. Surgery to remove all or most of the tumor is usually recommended for people with Craniopharyngioma. What type of operation will be performed depends on the location and size of your tumor.

Open Craniopharyngioma surgery (craniotomy) involves opening the skull to access the tumor. During minimally invasive Craniopharyngioma surgery (transsphenoidal procedure), special surgical instruments are inserted through your nose. The instruments pass through a natural corridor to the tumor without affecting the brain.

Whenever possible, surgeons remove the entire tumor. However, because there are often many sensitive and important structures nearby, doctors sometimes do not remove the entire tumor to ensure a good quality of life after surgery. In these cases, other treatments may be used after surgery.

Radiation therapy. External beam radiation therapy may be used after surgery to treat craniopharyngioma. This treatment uses powerful energy beams such as X-rays and protons to kill tumor cells. During external beam radiation therapy, you lie on a table while a machine targets the energy precisely at the tumor cells.

Special external beam radiation technology, such as proton beam therapy and intensity modulated radiation therapy (IMRT), allows doctors to carefully shape and target the radiation beam, thus providing treatment to tumor cells and sparing nearby healthy tissue.

In rare cases where the tumor has not touched the bundle of nerve fibers that transmit visual information from your eye to your brain (optic nerve), a type of radiation therapy called stereotactic radiosurgery may be recommended. Technically a type of radiation and not an operation, stereotactic radiosurgery focuses multiple beams of radiation at precise points to kill tumor cells.

Another type of radiation therapy called brachytherapy involves placing radioactive material directly inside the tumor where it can spread the tumor from within.

Chemotherapy. Chemotherapy is a drug treatment in which chemicals are used to kill tumor cells. Chemotherapy can be injected directly into the tumor so that the treatment reaches the target cells and does not damage nearby healthy tissue.

Treatment of papillary craniopharyngioma. An uncommon type of craniopharyngioma called papillary craniopharyngioma may respond to targeted therapy. Targeted therapy is a drug therapy that focuses on specific abnormalities that enable tumor cells to survive.

Almost all papillary craniopharyngioma cells contain a mutation in a gene called the BRAF gene. Targeted therapy for this mutation may be a treatment option. Special laboratory tests can reveal whether your craniopharyngioma contains papillary cells and whether these cells have a BRAF gene mutation.