Pituitary Tumors (Adenomas) in Children

Pituitary tumors are abnormal growths in the tissue of the pituitary gland, a pea-sized organ located in the center of the brain, between and behind the eyes. The pituitary gland produces and regulates the release of hormones that control growth, sexual development and function, metabolism and the body’s response to stress. Pituitary tumors can reduce or increase the production and release of these hormones. They can also put pressure on the nerves and brain tissue that surround the pituitary gland, including the optic nerve.

Pituitary tumors are called adenomas if they are benign, which means that they are not cancerous and do not spread to other parts of the body.

They are called carcinomas if they are malignant, which means that they can spread beyond the pituitary gland into the brain, spinal cord or other parts of the body.

Pituitary carcinomas are rare in adults and ever rarer in children. Pituitary adenomas in children and adolescents are categorized in three additional ways:

By size:

• Microadenomas are smaller than 1 centimeter across. Most pituitary adenomas are this size.
• Macroadenomas are 1 centimeter across or larger.

By their effect on hormone release:

• Functioning pituitary tumors increase the production of hormones. Most pituitary adenomas are functioning tumors, and their symptoms are due to the extra hormone production they cause.
• Nonfunctioning pituitary tumors do not produce hormones. They may put pressure on or damage the pituitary gland and reduce or block hormone secretions.

By the types of hormones affected. Pituitary adenomas may increase or suppress the release of specific hormones, including:

• Growth hormone, which helps control the body’s growth and metabolism. A pituitary adenoma that increases the release of growth hormone in a child can cause a condition known as gigantism. A tumor that suppresses the release of growth hormone can slow or stop a child’s growth
• Thyroid-stimulating hormone (TSH), which plays a role in regulating growth, body temperature and heart rate.
• Adrenocorticotropic hormone, which helps the body respond to stress.
• Sex-related hormones, including luteinizing hormone, follicle-stimulating hormone, gonadotropin and prolactin, each of which drives aspects of the development of sexual characteristics and functions in puberty and after.

In some cases, pituitary tumors are associated with inherited conditions that also cause other health problems. In many cases, a cause for pituitary tumor development cannot be found.

Researchers are working to understand whether pituitary tumors are caused by genetic mutations, hormonal abnormalities, or a combination of these and other factors.

Symptoms of pituitary tumors vary widely, depending on the tumor’s size, whether it is causing an increase or decrease in the release of hormones, and which hormones are affected.

Symptoms in children and adolescents may include:

• Headache
• Nausea and vomiting
• Confusion
• Dizziness
• Seizures
• Runny or drippy nose
• Some loss of vision
• Excessive growth or an abnormal slow-down of growth
• Joint pain
• Early or delayed puberty
• Less frequent menstrual periods
• Anxiety, irritability or depression
• Restlessness or listlessness
• Irregular heartbeat
• Shakiness
• Excessive sweating
• Trouble sleeping

Note that many of these symptoms, such as headache, listlessness, irritability and runny nose, are common in childhood. On their own, for short periods, these are generally not a cause for medical concern. It is when these minor symptoms continue or recur and appear with more unusual symptoms, such as vision loss, growth changes or abnormal timing of puberty, that you should consult your pediatrician, and a referral to a specialist may be suggested.

Your doctor will usually begin with a physical exam and with questions about any symptoms you may have noticed.

• Height and weight will be charted to look for changes in growth patterns.
• The doctor may check for any associated physical findings.
• A neurological exam may be done to check mental status, coordination, reflexes and muscle function.

If signs indicate the possibility of a pituitary tumor, additional tests may include:

• Eye test to check for loss of vision, including narrowing field of vision.
• Blood tests to check for high or low levels of hormones and blood sugar.
• Urine tests to measure hormone levels.
• Magnetic resonance imaging (MRI) or computerized tomography (CT) scan to get visual images of the pituitary gland, brain and spinal cord. Imaging tests may be repeated over time to find out whether or how quickly the tumor is growing.
• Biopsy, in which tissue is removed from the pituitary gland and examined under a microscope.

In many cases, endocrinologists may collaborate with neuro-oncologists in the diagnosis and management of patients with pituitary tumors.

Treatment for pituitary tumors depends on the nature of the tumor and the effect it is having on the body.

• Drug therapy may be used to control the over-production of specific hormones or to replace hormones that are being under-produced. Different drugs are used to address different hormone issues.
• Surgery may be done to remove the tumor if drug therapy is not effective or if the tumor’s growth is putting pressure on the optic nerve or surrounding brain tissue. Surgeons may reach the tumor through an incision in the upper lip or at the bottom of the nose, or by cutting through the skull.
• Radiation therapy may be used to kill tumor cells or to keep them from growing. Radiation therapy for a pituitary tumor must be narrowly targeted to minimize damage to nearby brain tissue.
• Neuro-oncology provides input regarding other potential treatments for some tumor types.

For most children and adolescents with pituitary adenomas, treatment is effective in removing or stopping the growth of the tumor and or in countering its hormonal effects. Early detection and treatment improves long-term outlook. Smaller tumors (< 1 cm), which are more common, are often more amenable to typical treatment approaches than the larger tumors. All of these tumors require careful ongoing follow-up.

Lifelong treatment or follow-up is generally needed.

• If the treatment has removed the tumor, follow-up tests and imaging will be needed periodically to make sure the tumor has not returned.
• If the tumor is not growing and is left in place, follow-up tests and imaging will be needed periodically to make sure the tumor has not begun to grow.
• Medication to replace or control hormone production may be needed if the function of the pituitary gland has been changed by the tumor.

Children with pituitary tumors often require care from many pediatric specialties.

The Neuroendocrine Center at Children’s Hospital of Philadelphia (CHOP) offers families a coordinated and multidisciplinary approach to treatment for neuroendocrine disorders. Our team combines the expertise of pediatric endocrinologists, neuro-oncologists, neuro-surgeons, neuro-ophthalmologists, neuro-radiologists and pathologists.

All our team members have vast experience in the treatment of complex neuroendocrine conditions.