Expert Opinion – Gynaecological Oncologist

 

Gynaecological Oncologist

Prof Andreas Obermair is a most experienced pelvic surgeon and gynaecological oncologist and specialises in complex surgery for gynaecological cancer and complex pelvic gynaecological surgery.

Professor Obermair is based in Brisbane, Queensland and conducts a surgery in the Sunshine Coast twice a month. He is also a volunteer member of the expert scientific advisory committee for LSA.

Andreas altered

We asked Professor Obermair to explain the specific gynaecological challenges and considerations facing women with Lynch syndrome. 

Misconceptions about Lynch syndrome

Lynch syndrome is an under-researched area and riddled with misconceptions that need to worry us because misconceptions can lead to misinformation, which can lead to patients and health professionals making wrong decisions that they may regret later.

Gynaecological cancers associated with Lynch syndrome

Women with Lynch syndrome have a 27 to 71% risk of endometrial cancer, compared to 3% of the general population. Women with Lynch also have a 3 to 14% risk of ovarian cancer, compared to 1.5% in the general population. The management of endometrial and ovarian cancer risks in women with Lynch syndrome includes risk-reducing prophylactic surgery, chemoprevention (use of medications to prevent cancer) and surveillance.

Diagnosis of Lynch syndrome

It would be infeasible to test the entire Australian population for Lynch. Instead, we recognise sentinel cancers. For example, if a patient (male or female) gets diagnosed with bowel cancer, the tumour should be tested and Lynch syndrome may be diagnosed. As a consequence, all first-degree relatives should be offered genetic testing and some of them will be found Lynch-positive. Preventative steps will minimise the cancer risk in Lynch carriers. Relatives who don’t have Lynch can be reassured that they do not have an elevated risk of developing Lynch syndrome-related cancers.

Missing the diagnosis of sentinel cancers will not only cause harm to the patient herself (she has a high risk of developing subsequent cancers that could have been prevented), but also to all of her first degree relatives who could also claim that they could have known earlier and prevented the development of cancers.

Until recently, gynaecological cancer surgeons only tested for Lynch if patients had a family history or upon a patient’s request. However, family history can be a poor indicator about Lynch syndrome and our research that 50% of patients with proven Lynch syndrome actually do not carry or know of a family history. Based on family history, we would miss identifying half of all Lynch-related sentinel cancers.

To address this weakness, all women freshly diagnosed with endometrial cancer, regardless of age or family history, should be offered evaluation for Lynch syndrome. To do that, the surgeon should request Lynch testing from the pathology lab after surgery, and testing can be performed on the samples the pathologist already have. Private health insurances normally cover those costs. No new samples need to be taken. A positive test will require a further, confirmatory test to be absolutely certain. In our institution we implemented routine Lynch testing for all endometrial cancer patients recently.

Our Queensland group has established this system in collaboration with the major pathology companies and we appreciate the good collaboration with them.

Treatments available and their effectiveness

Risk-reducing, prophylactic surgery has been shown to be the single most effective strategy to prevent gynaecological cancer. This means removing organs when they are disease-free and not yet affected. If surgery has been decided upon, Lynch patients should have a full hysterectomy with removal of uterus, cervix, fallopian tubes and ovaries. The timing of surgical intervention needs to be carefully considered as you will no longer be able to conceive. Laparoscopic hysterectomy is the gold standard to perform this operation.

Chemoprevention

If surgery is not yet an option, oral contraceptives are an effective chemopreventive (i.e. drug-related) option to minimise the risk of both endometrial and ovarian cancer.
Taking the oral contraceptive pill will decrease the risk of endometrial and ovarian cancer risk by 50%. Given the high risk to start with the remaining risk is still unacceptably high in the long-term.

The levonorgestrel intrauterine device (Mirena IUD) is sometimes prescribed to reduce the risk of Lynch-related endometrial cancer. The Mirena has shown effectiveness to treat endometrial cancer in obese and morbidly obese women diagnosed with endometrial cancer. Obesity increases blood oestrogen levels significantly. The Mirena is effective in many obese women because it slowly releases progestin, which is the antagonist to oestrogen, the cause for the development of some types of endometrial cancer. However, many Lynch patients are not obese and at this point in time, data on the prevention of endometrial cancer with Mirena IUD in Lynch patients are sparse because research into the effectiveness of the Mirena IUD in those patients is still lacking.

I recommend chemoprevention as a good way to bridge the time until risk-reducing, preventative surgery can be performed.

Surveillance

Cancer screening programs are designed to detect a cancer when it is small and easily treatable. Australia maintains a very successful cervical cancer screening program (PAP smear) that screens for cervical cancer and pre-cancerous changes. Unfortunately, PAP smears will not detect endometrial or ovarian cancer.

For endometrial or ovarian cancer there is no screening available. High-quality research has shown that ultrasound is unreliable to detect small cancers in the endometrium or in the ovaries. Some cancers are simply too small to be detected using this method. In the absence of screening alternatives, surveillance for endometrial cancer may be offered through endometrial sampling (Pipelle) but is considered unreliable because cancers can develop in between examinations.

Serum tumour markers (e.g., CA125, HE4) are not accurate enough to detect ovarian cancer. Up to one third of all early-stage ovarian cancers and a number of ovarian cancer types do not produce CA125. By contrast, a number of non-cancerous conditions come with elevations in CA125 (fibroids, adenomyosis, arthritis).

Risks and downsides of prophylactic surgery

Risks and possible complications of prophylactic surgery depend upon the type of hysterectomy performed and your health status.

Unfortunately, vaginal hysterectomy will not be feasible in Lynch patients because the pelvic and abdominal cavity needs to be inspected carefully and peritoneal washings need to be taken at the time of surgery. Neither can be done at vaginal surgery.

Open, abdominal hysterectomy is the least preferred surgical approach because it is associated with the highest incidence of pain and surgical complications.

Laparoscopic hysterectomy (keyhole surgery) is the safest approach for patients with Lynch syndrome and should be offered to all patients because its risk of postsurgical complications is 30% lower when compared to open, abdominal surgery.

Compared to open, abdominal hysterectomy, laparoscopic hysterectomy is also associated with less pain, less blood loss, shorter hospital stay (1 or 2 days compared to 5 days) and quicker recovery (2 weeks compared to 6 weeks).

Some surgical risks remain and include nausea and vomiting (a short-term, temporary side effect of anaesthetics); postsurgical pain (taking painkillers regularly for at least 10 days is critical); risk of injury to bowel, bladder, ureters, blood vessels/bleeding and nerves (2%); readmission to hospital (5%); return to the operating theatres for further unexpected surgery (2%); deep vein thrombosis (0.3%); infection (3% to 5%); constipation for a couple of weeks (a common side effect of painkillers); collection on top of the vagina (5%); and vaginal discharge for up to 6 weeks (50%).

Overall, between 90% and 92% of all patients will not develop any of the above mentioned surgical side effects.

Young women may experience psychological stress after surgery and may find it difficult to adopt to the new self. Generally, quality of life will return to normal or better levels after several weeks. 

Risk-reducing, preventative surgery is not an option for women who still desire to fall pregnant.

In premenopausal women, the onset of menopause and its medium and long-term consequences need to be considered.

  1. Loss of fertility
  2. Symptoms of oestrogen deficiency: Hot flushes, night sweats, vaginal dryness
  3. Emotional stress: reduced libido, depression, cognitive decline
  4. Metabolic consequences: Loss of bone density, increased risk of cardiovascular disease

Women who undergo menopause under the age of 45 years should consider Hormone Replacement Therapy (HRT). HRT appears to be safe and has not shown to reverse the beneficial effects of surgical menopause. Women who had a hysterectomy will require oestrogen only (without progesterone).

Costs associated with treatment

In Australia we have a two-tiered health system.

In the public system, all healthcare is free and patients do not need to pay. You will be treated by fully qualified staff or fully qualified staff will supervise your treatment. Downsides include:

  1. Patients are not entitled to select their specialist. That means that another doctor might see you for each visit (prior to surgery, at surgery, after surgery).
  2. Waiting times can be significant at some times.

In the private system, you have an arrangement with a health practitioner of your choice. You will be a able to search for the specialist who provides you with the best value. The main downside includes:

Top specialists often charge co-payments from patients because the reimbursement from health funds is sometimes very limited and doesn’t cover the expenses associated with a big medical practice. Every patient should be given a cost estimate detailing the expected out-of-pocket expenses prior to any surgery.

If you have any questions to the above, I’d be delighted to be contacted. “

 Prof Andreas Obermair, Brisbane

www.obermair.info

 

References

Lu K.H, Daniels M. Endometrial and ovarian cancer in women with Lynch syndrome: update in screening and prevention. Familial Cancer. 2013;12(2):273-277.

http://www.obermair.info/information/hysterectomy/