Familial Cancer Clinic Institute for Women’s Health 1st Floor, Maple House 124 Tottenham Court Road W1T 7DN Telephone: 020 7380 6912 Fax: 020 7380 6929 Email: michelle.johnson@ucl.ac.uk Familial Cancer Clinic Information Sheet Surgery to prevent endometrial and ovarian cancer in women with Lynch Syndrome Contents 1 Introduction …………………………………………………………….. 2 2 What are the genetic alterations associated with LS? ……………… 2 3 What is the risk of developing ovarian and endometrial cancer in your lifetime? ……………………………………………….. 4 3 What is risk reducing hysterectomy and bilateral salpingo-oophorectomy? ………………………………………………………… 3 5 Am I a suitable candidate for risk reducing surgery? ……………….. 4 6 What are the main advantages of having surgery? ………………… 4 7 Is there a chance that cancer can be found? ………………………… 4 8 What are the main disadvantages of risk reducing surgery? ……….. 4 9 Can the symptoms of the menopause be controlled? ………………. 5 10 Does the age at which I have TLH BSO matter? …………………….. 6 11 What does the surgery itself involve? …………………………………. 6 12 What are the risks of surgery? …………………………………………. 7 13 Can women die from surgery? …………………………………………. 8 14 Apart from surgery, what else can I do to manage my risk of endometrial and ovarian cancer? ……………………………………… 8 15 If I want to have this surgery how do I arrange it? …………………… 9 16 What happens next? …………………………………………………….. 9 17 How should I prepare for my admission to hospital for TLH BSO? …. 10 18 Sources of Further Information …………………………………………. 10 19 Familial Cancer Clinic Contact details ………………………………….. 11 UCL Hospitals is an NHS Trust incorporating the Eastman Dental Hospital, Elizabeth Garrett Anderson Hospital, Hospital for Tropical Diseases, The Heart Hospital, The Middlesex Hospital, National Hospital for Neurology & Neurosurgery, Royal London Homoeopathic Hospital and University College Hospital. 1. Introduction This leaflet will answer questions you may have about surgery to remove your womb (hysterectomy), ovaries and fallopian tubes (bilateral salpingo-oophorectomy). This is in order to prevent a cancer arising in the lining (endometrium) of the womb (uterus) or ovaries. You may be considering this operation because you have been found to be at increased risk of developing these cancers on the basis of your family history or genetic test results e.g. LS (Lynch Syndrome) or HNPCC (hereditary non polyposis colorectal cancer). Lynch Syndrome is a condition where families have a strong history of certain cancers including bowel, endometrium, ureter, stomach and ovary. Being aware of all the available facts will help you make an informed decision about whether this type of surgery is right for you. Approximately 6000 women in the UK develop endometrial cancer each year. The exact cause of endometrial (womb) cancer is not yet known. In low risk women (women who do not have an LS family history), it most commonly occurs between the ages of 50 and 64 and after the menopause. In high risk women endometrial cancer can affect women at a younger age – late 30s and 40s. Similarly, about 6600 women in the UK are diagnosed with ovarian cancer each year. The causes are not yet completely understood. In the low risk population, the risk of developing ovarian cancer is very low in young women and increases as women get older. In women who are at high risk because of LS, ovarian cancer can occur at a younger age. 2. What are the genetic alterations associated with LS? In some families, the increased risk of cancer is related to a genetic alteration that is passed down through the family. The genes associated with LS are MLH1, MSH2, PMS2 and MSH6. We can arrange testing for these genes for women in the high risk group if a blood or tumour sample can be obtained from a relative who has had cancer. If we find a gene alteration, then other members of the family can have a gene test to see if they carry the same alteration. You may already have had genetic testing for one of the LS genes. Other ‘high risk’ women may not have had genetic testing for various reasons. 2 3. What is the risk of developing ovarian and endometrial cancer in your lifetime? Your risk of developing either cancer depends upon your family history. Most women do not have a close relative with endometrial or ovarian cancer. They have a low risk of developing these cancers themselves A small number of women belong to Lynch Syndrome (LS) families. We think that 40-60 per cent of women carrying an LS gene alteration will develop endometrial cancer during their lifetime. This risk varies between research studies. Some show that the risk of endometrial cancer depends upon the gene that has the alteration. There is evidence that it may be lower in MLH1* and PMS2 carriers. Women from LS families are also at an increased risk of developing ovarian cancer. Between 7 and 12 per cent (approximately one in ten) of LS gene carriers may develop ovarian cancer during their life time. 4. What is risk reducing hysterectomy and bilateral salpingo-oophorectomy? Risk reducing hysterectomy and bilateral salpingo-oophorectomy is an operation to remove the womb, ovaries and fallopian tubes. We usually carry out this procedure through keyhole (laparoscopic surgery). This is called TLH (total laparoscopic hysterectomy) and BSO (bilateral salpingo-oophorectomy). Sometimes your surgeon may recommend an open hysterectomy (i.e. one involving a bikini-line cut in your tummy). 3 5. Am I a suitable candidate for risk reducing surgery? Yes, if you have seen a clinical genetics specialist. They will tell you if you are at high risk of developing endometrial and ovarian cancer on the basis of your family history or the results of genetic testing. You should be clear about your risk of developing these cancers before you go ahead with surgery. 6. What are the main advantages of having surgery? 1) It removes the future risk of endometrial and ovarian cancer. 2) Having your ovaries removed before the menopause decreases your risk of developing subsequent breast cancer. However, we do not think that LS increases breast cancer risk. 3) It prevents non-cancerous conditions of the womb and ovaries (e.g. fibroids or cysts). 7. Is there a chance that cancer can be found? Yes, occasionally a cancer is found when the tissue removed is looked at under the microscope. Small cancers are found in the lining of the womb in approximately 5 per cent (one in twenty) of women. The risk of finding a small ovarian cancer is less than the risk for endometrial cancer. If cancer is found, you may need more tests and/or surgery to find out if you need more treatment. 8. What are the main disadvantages of risk reducing surgery? 1. You cannot become pregnant after this surgery. 2. There is a small risk of complications associated with having surgery. These are explained in detail below. 3. You will go through the menopause (stop having periods) if this has not already happened. The average age of the natural menopause is 51 years. After the menopause you may have hot flushes, night sweats, mood swings, tiredness, irritability, vaginal dryness and loss of libido (sex-drive). This is due to the lack of the female hormone, oestrogen. We can treat this with HRT (hormone replacement therapy). However, some women may still have symptoms even on HRT. You may be at increased risk of osteoporosis (thinning of the bones) unless you take HRT until the 4 age of natural menopause. If you cannot take HRT you may be given another medicine to strengthen the bones. 4. The risk of heart disease and other conditions like Parkinson’s disease and dementia may be increased in women who have their ovaries removed before the natural menopause. These risks were higher in women who did not take HRT after their operation. HRT appears to decrease these risks. 5. Some studies found that women under 45 who go through menopause after surgery but do not take HRT may have a shorter life span than women who do not undergo this operation. However, most of the women studied did not have an increased risk of cancer and many did not take enough HRT. Therefore, we cannot be certain these results apply to women with LS, particularly if they take HRT after their operation. 6. Menopause is an important stage in a woman’s life. For most women there are many thoughts, feelings and expectations about the menopause. You may be concerned about what is going to happen physically, and what it means for you as a woman and a person. Women often do better through this period of their life when they have thought about what it might be like beforehand. This is especially true when you have chosen to have an early menopause, rather than have it arrive in its own time. We can offer help with the process of making your decision about surgery, and afterwards, if you experience problems with the menopause. There are psychological therapies which show promising results in helping you cope with the menopause. These therapies are available through our service. We have a separate leaflet on the menopause which talks more about this. 9. Can the symptoms of the menopause be controlled? Yes, HRT will control the symptoms of the menopause in most women. We recommend that women below 50 take HRT to minimise symptoms and prevent bone and heart disease. For some women there are medical reasons not to take HRT, such as a previous history of blood clots or breast cancer. A small number of women do not find HRT effective. At the moment we are not able to predict who will fall into this category. 5 There are alternatives to HRT but these do not benefit all women. These include drugs like clonidine, and venlafaxine. Cognitive behavioural therapy (available through our psychologist) and some herbal preparations can be effective. However, herbal remedies may contain plant hormones which act in a very similar way to HRT. It is important to be aware that taking HRT following surgery is different from taking HRT after you have had a natural menopause. • Women who have not reached the menopause at the time of surgery. If HRT is taken after removal of the ovaries it is replacing hormones that would have been naturally produced by the ovaries until the menopause. There is no evidence that use of HRT in this situation increases risk of breast cancer; in fact, it may reduce this risk. • Women who have reached the menopause. A woman is considered to have reached the menopause if she has not had a period for at least 12 months. HRT use for more than five years after the natural menopause may increase the risk of breast cancer. Sometimes the symptoms of the menopause may be so severe that the benefits of HRT outweigh this concern. 10. Does the age at which I have TLH BSO matter? You should not consider this operation if you have not completed your family. If you have, then age is an important factor when thinking about having this operation. The aim is to get a balance between:1. Decreasing the chance of getting cancer. In women with Lynch syndrome, the average age of diagnosis for endometrial cancer is 48 years and for ovarian cancer is 42-48 years. 2. Coping with the side effects of an early menopause. You should discuss the effects of loss of female hormones with your doctor and set out a clear plan to manage the symptoms that will result. You should ask your doctor if you have a medical condition that might prevent you from taking HRT. 11. What does the surgery itself involve? We carry out risk reducing surgery in one of two ways. The first method is keyhole surgery (laparoscopy). Three or four small cuts (called incisions) are made on your tummy (abdomen). These normally measure 0.5 to 1 cm in length. One 6 cut is hidden in the tummy button (umbilicus), another just above the bikini line and one on either side of the tummy. The surgeon then places a telescope in the tummy button incision. This sends pictures of your abdomen to a television screen so the surgeon can see inside. The surgeon then passes surgical instruments through the other incisions and removes the uterus, ovaries and fallopian tubes via the vagina. Finally, the top of the vagina (where the uterus was attached), is stitched together to prevent bleeding. This does not significantly reduce the length of the vagina. Washings are taken from the abdomen to make sure there are no abnormal cells. Sometimes the surgeon uses a more traditional open method, which involves a bikini line or an up and down (vertical) incision. In a small number of women (less than one in twenty) the surgeon in theatre might find that it is not be possible to use the telescope. In such situations, an open incision may be necessary. Reasons for this might be technical problems during the operation, obesity, scarring from previous operations, or bleeding during the procedure. Women who have had previous abdominal operations are more likely to need an open incision. The average hospital stay following keyhole surgery is two days and five days for open surgery. After keyhole surgery you can usually return to normal activity after four weeks (including driving). The average return to normal activity is six weeks after open surgery. 12. What are the risks of surgery? All surgery carries the risk of minor complications. Minor complications are those that have no long-term effects but may delay recovery. Wound infections, urine infections and a chesty cough are among the more common examples. Some women might need a blood transfusion or develop a blood clot DVT (deep vein thrombosis) in the leg (or at times even in the lung). Rarely, a woman develops a hernia at the wound site. This may happen some time after the operation. Serious complications that can occur during the operation include damage to the bowel, bladder, ureter (tube that carries urine from the kidneys to the bladder) or a blood vessel. These are very rare (e.g. the risk of bowel injury is less than 1 in 300). It is possible for injuries to go unnoticed at the time of surgery because the injury is small or it has not appeared on the television screen. Again, this is rare, but should it happen, you might need a second operation. 7 The list of possible complications of surgery is quite long, and so only the most common have been mentioned here. It is important to bear in mind that the vast majority of women do not experience any serious complications at all and have an uneventful operation and post-operative recovery. In addition, if your doctors feel that you fall into the high risk group for endometrial and ovarian cancer, then for most women, the risk of you developing a cancer without the surgery is greater than the risk of a complication. If you are concerned about any complications, please speak to the doctor and nurses on the ward, so they can give you more information. 13. Can women die from surgery? There is always a very small risk of death from any operation. This is more likely to occur in women who have significant medical problems before the operation. 14. Apart from surgery, what else can I do to manage my risk of endometrial and ovarian cancer? Ovulation is the production of ‘eggs’ by the ovaries. Preventing ovulation may offer protection from ovarian and endometrial cancer. OCP (oral contraceptive pill) use, pregnancy or breast feeding can have this effect on ovulation. When women in the general population (who have no family history of cancer) use the combined OCP for five years, their risk of developing both endometrial and ovarian cancer is reduced by up to a half. Further studies suggest that this is also true for high risk women. There may however be an increased risk of developing breast cancer on the pill . The Mirena intra-uterine contraceptive device (hormone coil) may reduce the risk of developing endometrial cancer. Research on this is not yet complete, so we don’t yet know definitely if this is true or not. Screening for endometrial and ovarian cancer may detect these diseases in their early stages in some women. However, the current situation is that we do not know if screening is effective. 8 Ovarian cancer: We are looking at two tests (ultrasound scanning and a blood test for a tumour marker called CA125) in research trials. These tests can detect some cases of ovarian cancer before they cause any symptoms. However, neither test has yet been shown to save lives. Furthermore, both the tests can be abnormal in women who do not have cancer of the ovary. There is a trial called UKFOCSS (UK Familial Ovarian Cancer Screening Study) that is trying to find out more about the effectiveness of screening in high risk women. Endometrial cancer: Screening (in addition to the ultrasound scan and CA125 described above) involves an outpatient hysteroscopy with endometrial sampling. This can be performed in clinic under local anaesthetic. Hysteroscopy is the use of a thin telescope to look inside your womb. The doctor takes a small sample (called a biopsy) from the lining of the womb. We do not yet know if screening works and research is still ongoing. There is a separate leaflet about endometrial and ovarian cancer screening that you can request using the contact details at the back of this booklet. 15. If I want to have this surgery how do I arrange it? You can contact the FCC (Familial Cancer Clinic) using the phone number or email on the back on this leaflet to ask for an appointment with a doctor. At that appointment the doctor will explain the surgery. They will discuss the benefits and risks to you in detail. You will have the opportunity to ask the doctor any questions you have about surgery, menopause or HRT issues. Once you have made a decision to go ahead with surgery, you will be asked to give your consent by signing a procedure consent form. Your name will then be added to the waiting list. The length of time patients may have to wait for their admission to hospital can change from time to time. On average it is three to four months at UCL (University College London) Hospitals. 16. What happens next? You will be informed by the admissions team of your date at least 4 weeks ahead of time. The team will also send you an appointment for pre-assessment (normally a minimum of 1 week before the surgery date). At pre-assessment you will see a nurse who will carry out test such as checking your blood pressure to make sure you are fit for surgery. You should bring any medications that you are taking with you to that appointment. 9 17. How should I prepare for my admission to hospital for TLH BSO? You will be asked not to eat or drink anything for some hours before you come in for your surgery. The admissions team will send you clear advice about this. Do bring your toiletries, night dress/pyjamas, dressing gown and slippers in to hospital with you. You will also need to bring all the medicines you normally take with you (even if you were asked by the preassessment nurse to stop taking any of them.) On the morning of the surgery, you will be seen by the anaesthetist and surgeon. You can ask them any last minute questions you may have about the surgery itself. Please use the space below to write down any further questions or concerns you may have so that you can discuss them with the doctor during your appointment. * MLH1 - lifetime risk 25 per cent in one study and PMS2 (lifetime risk 15 per cent) 18. Sources of Further Information For further information on ovarian cancer screening, please contact: • UK FOCSS Team Gynaecological Cancer Research Centre UCL Institute for Women’s’ Health 149 Tottenham Court Road London W1T 7DN UKFOCSS@ucl.ac.uk 10 For other enquiries related to endometrial and ovarian cancer, please contact: • CancerBacUp and Macmillan Cancer Support 0808 800 1234 www.cancerbacup.org.uk • Ovacome 0845 3710554 www.ovacome.org.uk Your local Regional Genetics Service  www.bshg.org.uk/genetic_centres/uk_genetic_centres.htm 19. Familial Cancer Clinic Contact details Clinical Nurse Specialist 020 7380 6912 Clinic Secretary 020 7380 6912 Email: Fax: 020 7380 6929 Clinic Location 11