Pituitary Tumors and Endoscopic Surgery Webcast August 13, 2008 Marvin Bergsneider, M.D. Introduction

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1 Pituitary Tumors and Endoscopic Surgery Webcast August 13, 2008 Marvin Bergsneider, M.D. Please remember the opinions expressed on Patient Power are not necessarily the views of UCLA Health System, our sponsors, partners or Patient Power. Our discussions are not a substitute for seeking medical advice or care from your own doctor. Please have this discussion you re your own doctor, that s how you ll get care that s most appropriate for you. Introduction Hello and welcome once again to Patient Power sponsored by UCLA Health System. I m Andrew Schorr. I m just so impressed with UCLA Health System because when any of us has a health concern we go to the doctor, to one of the clinics, or maybe the big medical center, and we can feel confident that we can get help. Often our need is pretty routine, but there are people who have much more unusual medical needs, and I m just blown away when I talk to UCLA specialists about the level of sophistication for some of the truly rare conditions. We re going to talk about one today and that is where someone has a benign tumor of the pituitary gland. Let s remind you of what the pituitary gland is. It s a small bean-shaped gland located at the base of your brain. It s sort of behind your nose and between your ears, and when there is a benign tumor there a whole variety of symptoms can happen depending upon whether the pituitary gland is able to do its job, whether there s pressure from the tumor on other structures and functions of the brain right there right behind your eyes as you can imagine, or whether the tumor itself is making your hormones either be; some hormones be too much or too little. In this program we re going to discuss all that with a leading expert, and that s Dr. Marvin Bergsneider, and he is Professor of Neurosurgery at UCLA, and he s a specialist in the surgical approaches as part of a multidisciplinary team helping people with pituitary tumors. Dr. Bergsneider thanks so much for being with us. Oh you re very welcome. Good morning. Signs and Symptoms of a Pituitary Gland Tumor Dr. Bergsneider, so there s a whole variety of symptoms that can suggest many things but among them just may be a benign tumor in the pituitary gland. Take us through what some of those kinds of symptoms are and maybe if you want to break it down as to how it relates to different types of tumors that may be in that gland. 1

2 Sure. As you mentioned the pituitary gland is situated in the back of the nose, and it s just below the optic nerves and a structure we call the chiasm where the optic nerves cross on their way back towards the brain. So if the tumor is large in that area it can compress the optic chiasm, and patients will often present to their physician or their eye doctor with complaints of visual loss, that they lose their peripheral vision. Now some tumors of the pituitary gland actually secrete too much of one particular hormone, and hormones are those chemicals in your body that control bodily functions. There are three hormones that are typically involved, and the most common hormone is prolactin. This is the hormone that new mothers have a high level of that causes their bodies to produce breast milk, and they also stop having their menstrual period, so a woman who does not have a new baby who stops having her menstrual period or has irregular periods and/or may produce breast milk often goes to her doctor, and this is a common way in which a prolactin-secreting tumor is discovered. In men who have prolactin-secreting tumors, they don t have breast milk production or menstrual periods, so they often come in with low libido or lack of sexual drive. So that s the most common hormone-producing tumor, and the hormone-producing tumors can be small or they can be large and also cause visual problems. The second most common hormone-producing tumor is one that actually produces an excess of growth hormone. If it occurs in childhood you get giants like Andre the Giant who had a pituitary tumor. If it comes in later age after you ve stopped growing, then you get very, very coarse features, and your body changes. You get very large, doughy hands, and your feet are growing, and your facial features start looking more like Cro-Magnon man, and it s quite disfiguring. That s called acromegaly. The more disabling kind but fortunately much less common is if you have too much cortisol being produced by your body caused by a pituitary tumor that s called Cushing disease, and that s very disabling. Patients have a lot of weight gain, very high blood pressure, diabetes problems. It s one of the hardest to diagnose. All of that is where the tumor actually has been producing the hormone. What about when you have the tumor itself is not letting the pituitary gland produce enough of what it s supposed to do? Exactly, so just as the same mechanism that the tumor can compress the optic nerve it can compress the normal gland, and then you have inadequate function of the pituitary gland. The most sensitive hormone that s affected is your sex hormones. So, again, in men they have a very low sex drive and they have a very low testosterone level. It affects women a little bit differently. When it s severe you can actually have an inadequate amount of thyroid level in your blood or even cortisol levels in your blood, and patients can get quite sick, but those are much less common fortunately. 2

3 Now all of that is where there s sort of a biochemical effect going on. You mentioned about blurry vision. So if the tumor is just there and not doing anything, all the levels are right, how would that show up? Would it be blurry vision? What other effects could it have if it s sort of squishing some things in the brain? Yes, actually your central vision is pretty good. So when you re looking straight at something you can see it just fine. What you lose is your peripheral vision. I remember a patient who came in saying that she kept watching The Price is Right and she couldn t see the outer letters of the; she was supposed to figure out what the word was until she came to the very; she finally couldn t do the show any more, and that s what brought her to the doctor. It s really the loss of the peripheral vision. For some patients they re discovered because they get into a car accident. They re driving along, and you use your peripheral vision when you drive to look for balls coming in from the side of the street or cars coming from the side of the street, and patient s get hit from the side because they never saw the car coming. Diagnostic Tests and Treatment Options Dr. Bergsneider, so all these effects can be seen in the body in a variety of ways. The bad actor if you will, is this nonmalignant tumor that is in the pituitary gland, this very small gland in a critical place in the brain. So if somebody is listening and they say, Well this sounds like me. It could be at work, how is it finally detected? What test do you need? I imagine there are blood tests as far as the biochemical effects, but beyond that how do you confirm what you re dealing with and that it s happening at the pituitary gland site? That s right. So the workup does involve blood tests usually ordered by an endocrinologist, and the second component is an imaging study, and the best imaging modality is an MRI scan; a dedicated pituitary-sequenced MRI scan; where they do very thin cuts to that very small area of the brain looking for a pituitary tumor. Okay so I would hope then, and I know at UCLA you have actually neuroradiologists who specialize in this. So then you know what you re dealing with; so then the question is what to do about it. Now, is drug therapy in some of those tumor situations a first line of approach? Yes. For the tumors that secrete prolactin it is one of the best therapies because the drug therapy is very effective and has very few side effects. For the other hormone-producing tumors, the growth hormone producing tumors, and the one that causes Cushing disease 3

4 with too much cortisol, the medical therapies aren t as effective and are used often to supplement a surgical approach if possible. For patients who have inadequate function, of course hormone replacement is part of the clinical management of the disorder. Let s talk about surgery because I know is so many of these cases then surgery comes into play. For many years there s been surgery through one nostril of the nose. At UCLA I know you take an approach where you go through both nostrils, and there s you as a neurosurgeon but there s also a head and neck surgery who works with you, a sinus endoscopic surgeon, and you have this endoscopic approach but still through the nose and as part of the workup, if you will, of the patient I know you have an endocrinologist who specializes in pituitary disorders to help you as well. So tell us about your team and then help us understand this surgery, this minimally invasive surgery, that you do at UCLA and how that s an option that people could consider. Sure. We ve really strived to make a comprehensive pituitary tumor clinic where a patient who comes in is able to see all the different experts because these are actually very complicated conditions to deal with; whether to do surgery, whether the timing of hormone replacement or drug therapy; so we have found that if you re able to talk among the different experts right there in clinic with the patient that decisions could be made, and hopefully we can educate the patient on why we re making a certain recommendation. In terms of the endoscopic approach, this combined teamwork approach really uses the combined expertise of a sinus endoscopic surgeon who really gets the neurosurgeon to the tumor. There are a lot of nuances in terms of the approach of removing a certain amount of tissue in the back of the nose and of bone and mucosa, and then once you get to the area that s overlying the tumor, then the neurosurgeon comes in. It s a threehanded surgery basically, and that s why it requires two people really to do it most effectively. When removing the tumor, the neurosurgeon wants to work with two hands independently in an area while the head and neck surgeon holds the endoscope and keeps moving the endoscope and keeping the field of view where the neurosurgeon needs to work, and that s why for the most important part of the procedure, at least from the tumor removal aspect, having two surgeons is really optimal. How s the recovery from that? Two surgeons working deep in the brain sounds like a lot. When someone is trying to recover from that, how quickly do people get to go home, and what have you found of the recovery? The recovery in terms of hospital stay varies from patient to patient. One of the factors that comes into play is when you re performing the surgery there s two main things that can happen that can hold someone in the hospital a little bit longer. One is that if it s a large tumor there s a chance that you can breach the outer capsule of what s holding the tumor back from the brain, and you could have cerebral spinal fluid leaking from the brain 4

5 area down through your nose. If that occurs, and it occurs in about 10-20% of the cases, then we have to reconstruct that area and sort of seal it up, and that may keep the patient in the hospital a little bit longer while we use other measures to keep the pressure off your closure. The other factor that comes into play is that when you manipulate around the normal gland, and sometimes a normal gland is very flattened out and very thinned out. When you take the tumor off of that some of the functions of the normal gland sort of goes to sleep for a short while, and the most common problem that occurs after surgery in that respect is that a patient will just make way too much watery urine, and it almost always comes back, but they may make cc of urine an hour, and in that case they can t go home right away. So if you don t have a spinal fluid leak and don t have an excessive amount of urine output, a patient could actually go home the next day or the following day following this surgery. And that s most often the case. Correct. Well, it s obvious that you have very sophisticated knowledge of this. So if someone wants to seek the expertise of you and your team, do they get referred there? Can they selfrefer, and how do you feel about second opinions because I know that your center is very specialized in this multidisciplinary treatment of this and the endonasal surgery that you do. The answer is yes, yes, and yes. We ll be happy to evaluate patients. We even do film reviews for patients who maybe live far away just to see if they re a candidate for coming down to have a visit. It would seem like for the patients listening then the first thing is understand is there a pituitary problem that s causing the symptoms they may be experiencing and then consult with specialists such as yourself to see well what s the best approach. Does that sound like a good treatment plan for people, a good approach? Absolutely. 5

6 Where does it come from, I wonder? So, is there some percentage of people where there actually is a history of these sorts of problems in their family or most of the times does it just come out of the blue? Most of the time it s out of the blue. There have been some genetic defects that have been discovered. There are rare cases where they occur in families, and there are specific disorders in which patients can have multiple different types of tumors in their body of which pituitary tumors are one of them, but for the most part in almost all the patients its just apparently just a random event. Dr. Bergsneider, you must be very proud of what you re doing in a very specialized area, and this team approach seems to allow you to really give people the care this population really needs and deserves. It must be very satisfying. We certainly try. Thank you so much. We ve been visiting with Dr. Marvin Bergsneider who s Professor of Neurosurgery at UCLA Health System, and I think this really illustrates the leading edge of treatment of not a common condition but one that causes all these symptoms for people even putting pressure on their vision and complications there as well and certainly something that happily can be dealt with in a minimally invasive way. As you heard, most of the time should people need surgery they get to go home right away, and then I understand that some of the time there could be follow-up radiation, but again you can count on UCLA to have very targeted approaches for that. This is what we do on Patient Power. Please look at all of our programs that are right there on the website. As we like to tell our listeners, knowledge can be the best medicine of all. I m Andrew Schorr. You ve been listening to Patient Power sponsored by UCLA Health System. Please remember the opinions expressed on Patient Power are not necessarily the views of UCLA Health System, our sponsors, partners or Patient Power. Our discussions are not a substitute for seeking medical advice or care from your own doctor. Please have this discussion you re your own doctor, that s how you ll get care that s most appropriate for you. 6

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