Midline Pituitary Tumor
Pituitary Tumors and Neuro-Endocrinology
Dr. Joseph Maroon along with their other UPMC colleagues work with the Departments of Neurosurgery, Endocrinology, Ear-Nose-and Throat, Radiology, Radiation Therapy and Neuro-Ophthalmology at UPMC for the evaluation, treatment and research of patients with pituitary tumors and disorders. The Specialists who make up this multidisciplinary program are leaders in their respective fields and have combined to make UPMC one of the most comprehensive programs in the country.UPMC is a referral center for the evaluation and treatment of pituitary tumors, pituitary adenomas, and tumors or disorders of the hypothalamic-pituitary region. Call, toll-free, at 888-234-4357 to learn more.
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Summary of Benefits of EEA To see surgical Video click – Here
The Endoscopic Endonasal Approach (EEA) is an innovative minimally invasive procedure that uses the nose and nasal cavities to access the skull base. This approach is ideal for tumors located in or near the pituitary gland. EEA enables surgeons to treat conditions in critical areas of the brain, skull base, and upper spine that were once considered to be inoperable or hard to reach.
The many benefits of using EEA instead of traditional open craniotomies include:
No incisions to heal
Faster recovery time
Fewer side effects
Reduced risk of neurologic damage
Endonasal Endoscopic Approaches to Pituitary Tumor – Longer Description The use of a minimally invasive endoscope had been used extensively in gynecology, laparoscopy (abdominal surgery), thoracic (chest), and cardiac surgery. This endoscopic technique of visualization functions on the principle of a flashlight rather than a cone of light. The microscope starts of as wide base that then focuses light to a narrow point creating a cone of light. This requires a wide exposure to deliver a small area of visualization ‘cone of light.’ In comparison, the endoscope starts of as a narrow light source that then widens to provide a ‘flashlight’ view.
In 1999 the skull base program at UPMC made a formal commitment to take advantage of this technology for accessing the skull base. Believing that if we could create a conduit that was narrow through the face we could deliver the flashlight to access the base of the skull and then progressively the brain. The nose provided the ideal conduit. Much work had been done in the ENT field in using the nose to access the region but had stopped short of the skull base. We now extended this to approach and resect tumors of the skull base and then of the brain itself. This approach would allow one to work from ‘inside out,’ i.e. start directly at the target and work outward. In the process it would not require the layers of facial skeletal removal that were common with conventional approaches. In fact there would be no need for incisions at all as the nose would provide the passage.
UPMC is now recognized as a Center of Excellence for the development of this technology. The Endonasal Approach allows extended reach to pituitary tumors and the ability to remove them completely through the nostril. The type of tumor approach allows surgeons to more easily deal with possible adhesions and other critical nerves and arteries in this part of the brain. In fact, this approach allows for a more targeted approach by starting directly at the tumor and working outward. In so doing it minimizes the manipulation and need to move these critical structures of the brain.
In essence the procedure allows both the approach and resection of the tumor to be done with minimal disturbance to the surrounding tissues. We no longer need to make incisions and disassemble the face so we can deliver a “cone of light” (microscope) to the skull base. Instead we can now deliver a ‘flashlight’ (endoscope/telescope) directly to the target via the nostril. This has the potential to reduce the impact on the patient, lessen the morbidity, and reduce the length of stay in many cases. Some procedures are now performed as overnight stays, with the patient, in some instances, returning to work in days.
INDEX of Diseases and Conditions Treated:
Pituitary Gland: Function and Diseases
- Background
- Acromegaly
- Gigantism
- Diabetes insipidus
- Galactorrhea
- Pituitary Tumors
- Treatments for Pituitary Dysfunction
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See large tumor in center
Pituitary Gland: Function and Diseases
Background:
The pituitary gland, or master gland, is located in a bony chamber at the base of the brain It is suspended by a stalk behind the sphenoid sinus located behind the nose. The pituitary gland is made of two lobes which control the many endocrine regulated growth and many other bodily functions. The large anterior (front) pituitary lobe controls the production of various bodily hormones, and the posterior (rear) lobe governs the body’s fluid balance and releases hormones made in the hypothalamus.
Frontal Lobe: The anterior lobe produces six hormones. Growth hormone, as its name implies, regulates the physical growth of most parts of your body. Prolactin stimulates the breasts to produce milk. The four other hormones made by the anterior lobe (thyroid-stimulating hormone (TSH), corticotropin (ACTH), follicle-stimulating hormone, and luteinizing hormone).
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Diseases of the Pituitary
Acromegaly
In this rare condition, the pituitary gland produces too much growth hormone, which causes excessive growth. However, an adult cannot grow taller, since the bones mature and vertical growth stops at the end of adolescence. Instead, the excess growth hormone produces acromegaly, in which your bones thicken and all other structures and organs grow larger. Overproduction of growth hormone is usually caused by a Pituitary tumor.
Acromegaly generally can occur at any time after adolescence. Usually, the first symptom is enlargement of the hands and feet, typically revealed by the tightening of a ring on the finger, or an increase in glove or shoe size.
After the hands and feet increase in size, the head and neck grow broader, and the lower jaw, brows, nose, and ears become prominent. The skin and tongue thicken, and features become generally coarser. The voice may become deeper.
Many people with this disorder experience tingling in the hands, fatigue, headaches, increased sweating, stiffness, and generalized aches. In a woman, the amount of hair on the body and limbs may increase.
In some cases of acromegaly, Diabetes mellitus develops and causes the symptoms of that disorder. If a large pituitary tumor is present, it may cause a number of other symptoms.
The longer acromegaly is left untreated, the greater the risks become. The heart continues to enlarge; High blood pressure and possibly Heart failure develop in time. In addition, a large pituitary tumor can cause serious problems with the eyesight. Also, certain cancers are more common in people who have acromegaly.
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Gigantism
Gigantism is the same disorder as Acromegaly, except that it begins in children and adolescents instead of in adults. The pituitary gland overproduces growth hormone, usually because of a Pituitary tumor. Overproduction of this hormone causes excessive growth of all parts of the body. The main difference between the two forms of the disease is that acromegaly occurs when the arm and leg bones have stopped growing, and gigantism occurs when they are still growing so that the young person grows to giant proportions. Treatment is the same as acromegaly.
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Diabetes insipidus
In the normal production of urine, your kidneys first filter water and other substances from your blood. The kidneys then reabsorb almost all of the filtered water, leaving urine ready to be excreted from your body. The absorbed water is returned to the bloodstream to maintain the correct concentrations of minerals, proteins, and other nutrients in blood and body fluids. If you lose a lot of body water because of fever, perspiration, vomiting, or diarrhea, for example, the kidneys reabsorb almost all of the filtered water and a small amount of concentrated urine is excreted. On the other hand, if you drink large amounts of water, the kidneys reabsorb less filtered water, and a large amount of dilute urine is produced. The process of water reabsorption is regulated by antidiuretic hormone (ADH), a hormone that is produced by the posterior lobe (rear part) of the pituitary gland. Diabetes insipidus is a disorder in which there is a deficiency of ADH, causing your body to pass large quantities of urine that contain a high percentage of water.
The most common cause of the disorder is damage to the pituitary gland from a severe head injury. The condition may also result from scarring or damage caused by an operation on the pituitary gland, or may be due to the effects of radiation therapy on the gland or on the surrounding area. Rarely, diabetes insipidus is caused by pressure on the posterior lobe of the gland from a pituitary tumor. In a form of the disorder called nephrogenic diabetes insipidus, the condition results from an insensitivity of the kidney tubules to ADH. In this condition the level of pituitary secretion of ADH is normal.
The main symptom of diabetes insipidus is that you pass large quantities of colorless urine, as much as 21 qt (20 liters) every 24 hours. This great fluid loss results in an unquenchable thirst. You will constantly be interrupted during the day and awakened at night by the strong need to urinate and drink. Potentially fatal dehydration can occur rapidly. Hormone tests to access ADH function are done by the Endocrinologist and will confirm the diagnosis.
The treatment of diabetes insipidus, is the use of a synthetic form of ADH, taken either as nose drops or by injection. The length of time you must take the drops or receive the injections is determined mainly by what has caused the disorder. If it was caused by a head injury, surgery, or radiation therapy, the defective gland may return to normal within a year, which means that you will be completely cured. If this fails to happen, you will probably have to take medication for the rest of your life. If the disorder is caused by a pituitary tumor, the tumor will probably have to be removed (see Surgical Treatments).
In the nephrogenic form of the disorder, you may only need to restrict your salt intake and take certain diuretics or other medication to help your kidneys conserve water.
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Galactorrhea
Galactorrhea is production of breast milk when it is not supposed to be produced. Milk production normally occurs in a woman a few days before, and in the months following, the birth of a baby. Production at any other time in a woman, and at any time in a man, is called galactorrhea. The problem is not a serious threat to health, although it may be annoying. However, the underlying cause of galactorrhea may be a pituitary tumor, which is more serious and may lead to other symptoms.
Galactorrhea is uncommon in women and rare in men. It is usually caused by excessive amounts of the female sex hormone estrogen, which can occur during pregnancy or from taking oral contraceptives or some other medications. Another possible cause is excessive production of the hormone prolactin. Prolactin is made by the pituitary gland and stimulates milk production. Galactorrhea can also be caused by a disorder of the pituitary gland such as a tumor or may occur for no apparent reason. In women, galactorrhea is often coupled with an Absence of periods.
In order to eliminate the possibility of a pituitary tumor or another underlying disorder the Endocrinologist will probably refer you for diagnostic tests, which may include a MRI scan and blood and urine tests.
If tests fail to reveal any cause for the condition, you will probably not need any treatment. If the problem is caused by drugs, discontinuing the drugs will clear it up. If galactorrhea is caused by a pituitary gland disorder or some other disease, the Endocrinologist may prescribe hormone treatment or the drug bromocriptine to prevent milk production. If the cause is a tumor you will be refereed to a Neurosurgeon for possible surgery (see Surgical Treatments).
Other Prolactin Disorders
Amenorrhea-Galactorrhea Syndrome; Drug-induced hyperprolactinemia; Infertility; Male hypogonadism.
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Pituitary Tumors
Your pituitary gland is divided into two parts, the anterior, or front, lobe and the posterior, or rear, lobe. Pituitary gland tumors almost always occur in the anterior lobe.
More than 10 percent of all tumors inside the skull develop in the pituitary gland. There are two main types. Pituitary adenomas are overgrowths of one or more of the cell types in the gland. These tumors are almost always benign. Most are less than ½ in (12 mm) in size. But because they grow in a confined space they may run out of room and may damage the nerves leading from the eyes, cause headaches, and eventually cause serious brain disorders if they begin to increase further in size.
About 50 percent of all pituitary adenomas secrete abnormal amounts of the hormone prolactin. This may cause no symptoms. Or it may result in impotence, absence of menstrual periods, or Galactorrhea. Pituitary adenomas may also secrete other hormones, and this can lead to Acromegaly, Gigantism, or Cushing’s syndrome. The tumor may also enlarge and press upon surrounding areas, causing other disorders. The causes of pituitary tumors are unknown. The incidence is 1 out of 10,000 people.
Types of Pituitary tumors
Prolactinomas; Crainopharngioma, Cushing’s disease; Acromegaly; Non-Secreting tumors; Recurrent Pituitary Tumors.
Common Signs and Symptoms:
Note: Symptoms are related to hormone deficiencies and pressure on intracranial structures.
Headache
Visual changes and loss
Seizures
Change or loss of menes
Personality changes
Impotence in men
Enlargement of hands, feet and/or facial features
Intolerance of heat or cold
Excessive thirst of urination
Skin changes
Loss of body hair
Breast discharge in women
Breast enlargement in men
Irritability
Testing:
CT scan of Brain
MRI Brain
Skull X-ray
Eye exam including Visual Fields
Angiogram
Endocrine function tests (blood work)
Growth hormone
TSH
Thyroid Hormone
Cortisol
Prolactin
ACTH
Craniopharyngioma:
The other type of tumor is called a craniopharyngioma. This type of tumor does not cause overproduction of any hormones, but it does progressively enlarge and can exert pressure either on the anterior lobe, which causes Hypopituitarism, or on the posterior lobe, which causes Diabetes insipidus. It can also press on the nerves to your eyes and, as a result, eventually cause headaches, double vision, and deteriorating sight.
- tumor located near the pituitary stalk
- often benign, but close to vital structure making surgical removal difficult
- rare, less than 5% of childhood brain tumors; average age is 7 – 12 years old
- presenting signs include vision changes, headache, , changes
- treated with combination therapy, usually surgery and radiation therapy; there is some controversy over the optimal approach to therapy
- survival and cure rates are favorable, though endocrine dysfunction may persist as well as the effects of radiation on cognition (thinking ability)
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Treatments for Pituitary Dysfunction
Once recognized, these patients require Endocrinologist evaluation and blood sample will be taken to find out how much growth hormone is circulating in the body. CT or MRI scans will definitely show the extent of the tumor. Tests of your visual field (side vision) will also be required.
If the blood test shows that the growth hormone level is high and cannot be reduced with medications, and if the presence of a tumor is confirmed by CT or MRI scans, you should be treated as soon as possible. The Endocrinologist will recommend either radiation therapy or surgery.
The best form of treatment depends on a number of factors. Drugs such as bromocriptine can sometimes control a pituitary tumor and are often used first. The drug somatostatin is often used to treat acromegaly; it inhibits growth hormone, which in turn checks the growth of the pituitary tumor.
Surgical removal of the pituitary tumor may be effective in improving problems with the eyesight. Also, tumors can now be selectively removed, leaving behind only healthy tissue. Treatment often halts the progress of the disease. Cosmetic recovery is often possible, and changes in your bones and your appearance may be reversible. (see Top section on EEA surgery)
If all or almost all of your pituitary gland is removed or destroyed, you may need lifelong treatment with hormone replacements, such as sex hormones or thyroid hormone.
Possible Risks
Certain risks must be considered with any surgery: infection, excessive bleeding (hemorrhage), and an adverse reaction to anesthesia. Since pituitary tumor surgery involves the brain, other risks include meningitis and the leakage of cerebrospinal fluid (CSF) from the operation site. Due to the proximity of the optic nerves, visual blurring can occur. Risks of surgery will be reviewed by your neurosurgeon.
Post-op Recovery
The outlook for recovery without physical limitation after this procedure is excellent. If partial vision or visual field loss has occurred, its progress can usually be arrested and often reversed. If the tumor is completely removed, recurrence is unlikely. Recover of hormone function is often immediate but can be delayed and may require the use of short term or long term hormone replacements.
Tri-State Neurosurgical Associates-UPMC
Office Addresses:
Administrative Oakland Office
Presbyterian University Hospital
Department of Neurosurgery
Suite 5C
200 Lothrop Street
Pittsburgh, PA 15213
Phone: 1-888-234-4357
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