And It's About Time There Was Some Support For Cushing's!
Chief, Neurosurgery Service/Director, Neuro-Oncology Research Laboratory/NABTT (new Approaches to Brain Tumor Therapy) Investigator/Program Leader, Neuro-Oncology Program
H. Lee Moffitt Cancer Center & Research Institute
12902 Magnolia Drive
Tampa, FL 33612
College / Department /
-Medicine / Interdisciplinary Oncology / Clinical
-Medicine / Neurology /
Education & Training:
- M.D., Harvard Medical School, Boston, MA, 1972
- Internship, Massachusetts General Hospital, 1973
- Residency, Massachusetts General Hospital, 1981
- Fellow, New York Hospital, Cornell Medical Center, 1980
Clinical & Research
Frameless image-guided surgery, surgery of brain metastasis and malignant tumors, experimental therapeutics, anti-angiogenesis therapies, pituitary surgery, skull base surgery
Neurosurgical Advances in the Management of Pituitary Tumors
Steven Brem, M.D.
Program Leader, Neuro-Oncology Program
H. Lee Moffitt Cancer Center & Research Institute
Professor of Neurosurgery
University of South Florida College of Medicine
Length: 35 minutes
Speaker Disclosure: None
Target Audience: This program is intended for physicians and nurses who are primarily involved in cancer care, research and education and is intended to advance clinical and research reasoning to contribute to the mission of the prevention and cure of cancer.
Click on this link to view his presentation for free. Once on the link go to the bottom of the page and click on the (View Presentation for Free) http://network.moffitt.usf.edu/eventdetails.asp?EventID=212
Just some more info. Dr. Rhoton,Jr. has retired and Dr. Steven N. Roper has the ball now. This Doctor and the University of Florida are listed on the PNA. And they do not pay to be listed on this site. The link is http://www.pituitary.org/memberdirectory/
University of Florida Department of Neurosurgery
You have been referred to Dr. Albert L. Rhoton, Jr., at the University of Florida Clinic in Gainesville for evaluation of a pituitary tumor and to assist you in selecting the best method of treatment. Treatment of your pituitary tumor may include drug and/or radiation therapy, surgery, or a combination of these treatments. There are also some tumors which are best observed without treatment because of their very slow growth. This booklet was prepared to assist you in selecting from among the various treatment alternatives. Following treatment you will be seen at regular intervals to monitor your condition.What is the Pituitary Gland?
The pituitary gland, located at the base of the brain, is a small organ about the size of an acorn. It is surrounded by a bony saddle-like structure above the sinuses at the back of the nose, called the sella turcia. The pituitary gland is sometimes referred to as the "master gland" because it releases substances which control the basic functions of growth, metabolism, and reproduction.
The pituitary gland is divided into two parts called lobes. These are referred to as the anterior and posterior lobes. Each lobe releases special substances, or hormones which control basic activities within the body. The specific hormones and their activities are shown in Figure 1. (See Figure 1)
A tumor in the pituitary gland causes symptoms by either releasing too much of a hormone or by pressing on the gland causing it to release too little hormone. The symptoms one experiences from the pituitary tumor are often determined by the type of tumor. A tumor that secretes hormones produces symptoms by releasing too much of the hormone. Some tumors cause the gland to stop releasing enough hormones. In this case symptoms arise from lack of hormones. A pituitary tumor may also cause symptoms by growing and pressing on the structures, like the nerves to the eyes, surrounding the pituitary gland.
A pituitary tumor can cause an increase in any of the anterior pituitary hormones listed in Table 1. (See Table 1) For example, you may experience the following:
An increase in the amount of growth hormone (GH) causes the body to growat an abnormally fast rate. Bones become thicker, the hands and feet may appear wider or thicker, and the jaw may protrude.
If a tumor releases adrenocorticotropic hormone (ACTH), it causes increased cortisol which leads to fat deposits, especially in the shoulders and face.
Elevated levels of prolactin may cause secretion of breast fluids and decreased sexual drive in men and women. It may also cause irregular or absent menstrual periods in women, and difficulty in having an erection or infertility in men.
For pituitary tumors that do not secrete hormones, the initial symptoms may not occur until the tumor presses on the structures surrounding the normal pituitary gland.
|Thyroid Stimulating Hormone (TSH)||Causes the thyroid gland to grow and release thyroid hormones (called T4 and T3)|
|Adrenocorticotropic Hormone (ACTH)||Causes adrenal gland to release several hormones. The major one is cortisol.|
|Growth Hormones (GH)||The main hormone for general body growth|
|Follicle Stimulating Hormone (FSH)||Stimulates ovulation in women and the production of sperm in men.|
|Luteinizing Hormone (LH)||Stimulates ovulation in women and testosterone production in men.|
|Prolactin||Responsible for breast milk production in females.|
|Antidiuretic Hormone (ADH)||Controls thirst and the amount of urine produced by the kidneys.|
|Oxytocin||Stimulates uterine contractions in women|
Because the pituitary gland is located within the skull, when it becomes large, it may cause headaches that get worse as the tumor grows. Also, since the pituitary gland is located near the eyes, it can press on the nerves to the eyes and cause loss of vision. Loss of peripheral vision (See Figure 2) may occur first and be undetected by you. This may progress to eventual blindness if the pressure is not removed from the nerve. The tumor may also press on the nerves that move the eye and cause double vision. If the tumor is very large it may press on other parts of the brain and cause problems with memory, weakness, or numbness.
Because the pituitary gland affects many systems, several kinds of doctors may be involved in your care. These doctors are medical specialists who will recommend certain tests to evaluate the tumor based on your symptoms. Special tests called CT and MRI scans will be ordered to evaluate the size of the pituitary tumor. These are similar to X-rays. Neither of these scans is painful or requires special preparation. An ophthalmologist may be asked to do visual field tests to evaluate any visual problems caused by the tumor. An endocrinologist is the hormone expert involved in your care. The endocrinologist will monitor the hormone levels in your blood and urine and make recommendations based on the results of these tests.Most pituitary tumors can be cured with the treatments that are available. There are four different types of treatment approaches which can be taken after a pituitary tumor has been diagnosed. These are:
Some pituitary tumors may be observed without treatment because they may grow very slowly. Pituitary tumors are usually benign. It is rare for them to ever become malignant. If a decision is made to observe the tumor without treatment, ongoing evaluations by CT or MRI, by an endocrinologist, and by an ophthalmologist will be done. CT or MRI scans will be performed within 3-6 months after the initial diagnosis and every 6-12 months thereafter until the situation has been clarified. This period of observation without treatment provides information that helps the doctors decide whether other treatments that have more risks are needed.
Observation without treatment may also be recommended if conditions are present that would seriously increase the risk of surgical or other treatments. Because pituitary tumors are slow growing, patients can often be observed without treatment for long periods of time without the tumor causing serious problems. This is often the recommended form of treatment for patients who are age 70 or above or who have a serious medical illness such as heart disease. Close follow-up may be necessary to monitor tumor growth and symptoms.
Only one type of pituitary tumor can be successfully treated with drugs. This type of tumor secretes a hormone called prolactin. Tumors that secrete prolactin are called prolactinomas. Prolactinomas can often by controlled by a drug called Bromocriptine. This drug will most often cause a reduction in the size of the tumor and at the same time cause a decrease in an abnormally high prolactin level in the blood. Although Bromocriptine may control the tumor, it may not cure it. Treatment with Bromocriptine must often be continued for many years at reduced levels.
In most cases, Bromocriptine is an effective treatment for prolactin secreting tumors and has few side effects. However, some patients develop nausea, headache, dizziness and weakness while on this medication. Usually these symptoms can be reduced by taking small doses of the drug at first and slowly increasing the dosage up to the needed level over a period of several weeks or months. Bromocriptine will not control all prolactin-secreting tumors. For approximately 20 per cent of patients with prolactinomas, surgery or radiation therapy may be needed.
Another treatment option for pituitary tumors is radiation therapy. Radiation therapy is most commonly used after surgery. Most people think of radiation therapy as a treatment for malignant tumors. As previously mentioned, most pituitary tumors are benign tumors and not malignant. Even though most pituitary tumors are benign, some tumors have roots in the bone or coverings around the brain that involve vital nerves or blood vessels in such a way that surgical removal would cause significant damage to these important structures. In these cases it is best to treat the main part of the tumor with surgery and to use radiation therapy for the roots or remaining tissue.
Radiation therapy, given under the direction of specialist called a Radiotherapist, is most effective when the size of the tumor has been reduced by surgery. The greater the amount of tumor at the time of radiation therapy, the less effective the treatment will be. Radiation therapy usually requires a series of treatments lasting a few minutes each day for four to six weeks. In most cases, the treatment is painless and can be done on an outpatient basis. Radiation treatments are generally begun a few weeks or months after surgery to allow time for the incisions from surgery to heal. The ability of the gland to produce hormones often decreases slowly after radiation therapy. Twenty (20) percent of patients who have normal pituitary function early after the completion of radiation therapy will require hormone replacement after two years. This figure rises to 50 percent after five years.
A specialized form of radiation therapy called "radiosurgery" can be used in selected cases. Radiosurgery involves a single, highly focused radiation treatment. The patient can often return home after the single treatment which is done as an outpatient.
Surgery is the preferred method of treatment for most pituitary tumors. Two types of operations are done for the removal of pituitary tumors. One, called a craniotomy, is directed through the skull above the eye. The other, called a transsphenoidal operation, is directed through the nose. The craniotomy operation involves making an incision on the scalp near the top of the head. A piece of bone is then lifted out and the coverings over the brain are opened. The lower part of the brain is gently lifted to expose and remove the tumor. The piece of bone is then replaced and the scalp is closed with stitches or staples. In most cases, the incision on the head can be placed so that the scar is hidden by the hair. This type of operation is not often needed for a pituitary tumor, but it is sometimes necessary if the tumor is very large and/or it cannot be reached through the nose by a transsphenoidal operation.
The transsphenoidal operation is the most common operation for a pituitary tumor. The surgical approach for this operation is through the nose. There is no incision on the face. This surgical approach provides the best exposure of the tumor at the lowest risk. The operation normally takes two or three hours. Following the operation, most patients spend one day in the intermediate or intensive care unit before returning to their hospital room. Patients usually stay in the hospital for three or four days following the operation.
Certain risks exist with both the craniotomy and the transsphenoidal operation. With either operation there is a small risk to life as occurs with any anesthesia and major surgery. The risk to life is less than one percent. With either operation there is a risk to developing problems with vision because the nerves to the eyes are located in the area of the tumor. When there has been a distinct loss of vision before the operation due to pressure from the tumor, vision is often greatly improved by the operation. The degree of recovery of vision after the operation depends on how much damage has been done to the nerves of the eye by the tumor before the operation. If the degree of visual loss is minor before surgery, there may be full recovery. On the other hand, if there has been a marked loss of vision before surgery, there is often improvement in vision but not full recovery. It is possible but uncommon for patients who have not had a visual problem before the operation to have this type of problem afterwards.
There is some risk that surgery may damage the pituitary gland. In many cases pressure by the tumor has already damaged the gland. The chance of surgery damaging the gland is small if the tumor is small, however the risk increases when the tumor is large. In most cases, even with very large tumors, the gland regains normal function after a recovery period.
Another risk is a condition called Diabetes Insipidus which is caused by a decrease in antidiuretic hormone (ADH). A lack of this hormone leads to increased thirst and frequency of urination. The pressure from a pituitary tumor or surgery may cause this problem. Diabetes Insipidus can be treated by replacing the antidiuretic hormone with medication. This is usually given in the evening to reduce the frequency of urination during the night. Diabetes insipidus resolves in a few days in most patients.
Other risks associated with surgical intervention include double vision, numbness of the face, bleeding, infection, stroke, or other neurological defects.
At the initial evaluation, Dr. Rhoton will examine you and ask you questions about your medical history. If surgery is needed, a preoperative evaluation will be done. This evaluation often includes a general physical exam, EKG, chest x-ray, selected blood tests and a visit with the anesthesiologist. The preoperative evaluation is usually completed in the clinic the day before surgery, but it can be done as long as several weeks before the operation. Some of the tests can be done near your home if that will be more convenient
You will be admitted to the hospital on the day of your surgery. The night before surgery, eating or drinking is not permitted after midnight. After you arrive at the hospital on the day of surgery you will be taken to the preoperative "holding" room where intravenous (through the vein) fluids will be started and you will be given medication to help you relax. You will then be taken to the operating room where you will be moved to another bed and the anesthesiologist will put you to sleep.
Most pituitary tumors are removed by the surgical procedure called a transsphenoidal operation. Transsphenoidal means the operation is directed through the sphenoid bone and sphenoid sinus. The sphenoid is a small bone in the back of your nose located just below the pituitary gland. It often contains a large air filled cavity called the sphenoid sinus. In the past the transsphenoidal operation was begun by making a 1-2 inch incision under your lip at the top of your upper gum in the sublabial modification of the transsphenoidal approach (See Figure 3A) or within the nose in the transseptal modification. (See Figure 3B). In recent years Dr. Rhoton has modified the operation so that there is no need for an incision under the lip or in the front part of the nose in most patients. The new procedure is called an endonasal procedure because the tumor is approached through the nasal cavity without an incision under the lip or in the front part of the nose. There is no incision on the face. The tumor is reached by working through one nostril and making a hole at the back of the nose (See Figure 3C.) into the sphenoid sinus and through the layer of bone between the sphenoid sinus and the pituitary gland. The tumor is then removed. The endonasal procedure reduces the time required in the operating room by as much as 2 hours. In the older approach the incision in the mouth and elevating the mucosa from both sides of the nasal septum increased the length of the time required to expose the tumor and to close the area after the tumor is removed. The larger incision in the mouth also slowed the return of the patient to a normal diet and was associated with some risk to the upper teeth. The newer approach has proven to be as effective as the older approach in reaching the pituitary gland, and the benefits are greater. Patients can resume a normal diet sooner after surgery, and the swelling of the face and risk of injury to the upper lip and teeth is markedly reduced. Post-operative discomfort is decreased and hospitalization has been shortened by as much as two days.
Figure 3. (A,B,C)
A small piece of fat will be removed from just below the skin on your abdomen to fill the cavity created by the tumor removal. The bottom of the skull is closed with a piece of bone from the wall of the sphenoid sinus. (See Figure 4) This will help to prevent leakage of cerebrospinal fluid (CSF). The CSF is a fluid that surrounds the brain, spinal cord, and pituitary gland. This fluid acts as a cushion and provides nutrients for these structures.
In a small number of patients the fat packing will not hold and CSF may leak from the nose. If the drainage continues it may lead to the entrance of bacteria into the CSF. This may result in an infection called meningitis. Sometimes waiting a few days will allow this drainage to stop and no further treatment is needed. However, in a few patients (less than 1 in 100), another operation may be necessary to seal the opening at the base of the skull.
In the endonasal procedure there is no need for stitches to close the area since no incision has been made in the nose and mouth. There is no need for gauze packing in the nose as is used when incisions have been made in the nose or mouth.
What to expect After Surgery
After surgery, you will be transferred to the recovery room for several hours before being transferred to the intermediate or intensive care unit where you will remain overnight. In each of these areas, you may be connected to one or more monitors so that your condition can be watched closely.
Because the pituitary gland is at the base of the brain, an important part of your care after surgery is the frequent neurological checks by your doctors and nurses. You will be asked a series of questions that help to determine how well the brain is functioning. These checks will include some questions for you to answer (e.g. "What is your name?", "Where are you right now?", "Can you tell me the date?"). They will also shine a light in your eyes and test the strength in your arms and legs. These checks are necessary in order to detect and monitor any changes in your condition.
Monitoring Your Fluid Balance
As mentioned earlier in this booklet, your pituitary gland secretes a hormone called antidiuretic hormone (ADH) which regulates the fluid in your body. After surgery, the amount of ADH circulating in the body may decrease. The decrease in ADH will cause the kidneys to release water from the body, producing large quantities of dilute urine. Also, the large loss of water from the body may produce dehydration, and therefore, more fluids will be needed.
The deficiency of ADH is called Diabetes Insipitus. In most cases the deficiency resolves by the time you leave the hospital. The nursing and medical staff will be closely measuring both fluid intake and output. Therefore, it is important to keep an accurate count of every thing you drink as well as your urine output. As your recovery progresses you will need to continue to notify the nursing staff of everything you drink. A catheter (rubber tube) will be inserted into the bladder during surgery. This catheter gives an accurate measurement of urine output and is usually removed the morning after surgery. After the catheter is removed, your urine will continue to be measured by having you urinate into a collection container (called a "hat") which will be placed on the toilet in your bathroom.
During the first one or two days, an intravenous tube will remain in place until the medical and nursing staff feel you are taking enough fluid by mouth. The intravenous tube is also used to give antibiotics which help protect against infections.
Your Diet After Surgery
The nursing staff will keep fluids at your bedside and encourage you to drink when you are thirsty. The initial diet after surgery will be clear liquids. Certain drinks which contain a lot of salt (such as Gatorade) should be avoided. Using a straw is also discouraged as this will increase the pressure in the surgical area. The nursing staff will provide you with ointment for your lips if dryness is a problem. You will progress to your regular diet when you tolerate fluids well.
As mentioned previously under the description of the operation, a small piece of fat is removed from just under the skin over the abdomen to "plug" the opening in the back of your nose. The medial and nursing staff will also be monitoring the drainage from your nose. This is done by changing the pad covering your nose and monitoring the drainage for cerebrospinal fluid (CSF) which would indicate a leak (See Figure 5). You will also have a small dressing on your abdomen where the "plug" of fat was removed. The stitches from this incision will dissolve by themselves and do not need to be removed.Pain After Surgery
Usually the severity of pain after surgery is relatively mild. You may experience a headache which can be treated with medication. You may have some bruising under your eyes and/or along the side of your nose. This should resolve by itself within several days.
ActivityInitially you will be on bed rest. The head of your bed must remain in an elevated position to prevent swelling to the surgical area and help you breath easier. While you are on bedrest, you will wear a special type of stockings to improve the circulation in your legs. When you begin to get out of bed, usually the day after surgery, you will require assistance from the nursing staff until it is determined that you can walk safely by yourself.
As with any major surgery where you receive general anesthesia, it will be important to exercise your lungs to prevent complications. Deep breathing and the use of breathing devise called an incentive spirometer will help to keep your lungs expanded. Vigorous coughing or sneezing is discouraged to avoid an increase in pressure in the surgical area.
Recovering From Your Surgery
In most patients the body will adapt to the changing levels of antidiuretic hormone (ADH) and maintain a reasonable balance of fluid. However, for a small number of patients urine production remains too high. If this occurs, your doctors may begin temporary treatment with an injection of synthetic ADH. If the body continues to be unable to control urine production, you may need to take the ADH hormone after you leave the hospital. If this occurs, you will probably be given a form of ADH which can be taken as nose drops or spray, or a tablet.
During the weeks or months after surgery repeat hormone tests and eye exams are commonly needed. These tests will be similar to the ones that were done before the surgery. Often these tests can be done by the doctors in your community The endocrinologist will test how well the pituitary gland is functioning by checking hormone levels in the blood. If the levels are not high enough for everyday activities the doctor may prescribe hormone replacement therapy. The ophthalmologist will evaluate any changes in your vision after the surgery. Frequently, after the pressure from the tumor is relieved, visual problems will improve.
Discharge InstructionsDuring the first six or eight weeks, you should avoid sneezing and using any inhalants that might irritate your nose. You may gradually increase your activity level over the next three weeks until you reach your previous activity level. Most patients should plan on taking approximately two or three weeks off from work, although some patients may return earlier.
If your tumor is small you will probably not have to take any hormone replacement after you are discharged from the hospital. Patients with decreased pituitary gland function can still lead a normal life by taking medication each day to replace the normal hormones in the body. If your tumor is large you may be given a medication called Hydrocortisone. This medication helps to protect your body from the effects of stress. If you need hydrocortisone after discharge, you may be instructed to increase the dose if you are stressed by fever, infection, surgery, or other illness. Specific instructions about how to take hydrocortisone will be given to you before you leave the hospital.
Other medications that may be needed for decreased pituitary function are thyroid medications; estrogen or progesterone in the female; and testosterone in the male
A follow-up appointment will be scheduled with your neurosurgeon approximately six weeks after surgery. Laboratory tests will be done at that time to check the hormone levels in your blood and urine. Some patients develop a low sodium level in the blood during the first week or two after surgery because the pituitary gland over compensates by producing too much antidiuretic hormone (ADH). The excess ADH causes the body to retain fluid thus diluting the serum sodium to low levels. The treatment for this is fluid restriction and replacement of sodium either by salt tablets or intravenously. For that reason you will be asked to obtain several sodium or electrolyte levels after leaving the hospital. A prescription that can be given to a laboratory in your home area will be given to you before discharge from the hospital.
The decision to use radiation therapy is usually made in the hospital after surgery or at the time of a MRI several months later. In most cases, a follow up appointment will be scheduled four to six months after the first follow-up appointment done approximately six week after surgery. The MRI scan is usually repeated at this time. If there are any visual changes due to the tumor, you should also see the ophthalmologist for studies of the visual fields. Laboratory tests may be obtained at this time and your medications will be reviewed. The fact that some pituitary tumors recur emphasizes the importance of follow-up visits with your doctors. Most patients with a pituitary tumor resume a normal life after treatment. Vision usually improves if it was affected and fertility improves in many patients who were infertile.