Gynaecological sarcomas, sometimes shortened to gynae sarcomas, occur in the female reproductive system: the uterus (womb), endometrium (womb lining) ovaries, vagina, vulva and fallopian tubes. They can affect women of any age although they are very rare in women under the age of 30.
Most gynaecological sarcomas (85%) occur in the uterus (womb) and 7% occur in the ovaries. The remainder occur less commonly in the vagina, vulva, fallopian tubes and other areas of the female reproductive system.
Malignant Mixed Mullerian Tumour or carcinosarcoma is a mixture of carcinoma and sarcoma, where the carcinoma component is the dominant feature. These types of cancer are not treated as a sarcoma but treated in a way that is similar to other cancers of the ovaries and uterus.
Signs and symptoms
Symptoms of gynaecological sarcomas can vary depending on the size and location of the tumour. They may include:
- Heavy periods or bleeding in between periods
- An enlarging fibroid
- Vaginal bleeding after the menopause
- Blood in vaginal discharge
- Abdominal pain and bloating
- A noticeable lump on a section of the vulva
Symptoms can be confused with more common gynaecological conditions for example, problems with contraceptive devices such as the coil, menopause, post menopause symptoms, and fibroids. Most women with these symptoms will be referred to a gynaecologist by their GP.
How are gynaecological sarcomas diagnosed?
A diagnosis of gynaecological sarcoma may start with a visit to your GP who will then refer you to a specialist doctor, usually a gynaecologist. Your symptoms will be investigated further using a series of tests that may identify sarcoma. Tests may include:
• Clinical examination – looking at or feeling any lump
• A scan – taking pictures of the inside of the body using ultrasound, x-ray, CT, hysteroscopy or MRI
Some uterine sarcomas can be diagnosed from pictures taken during scans; however, many gynae sarcomas may also be found after or during a routine operation, such as surgery to remove fibroids or a hysterectomy. Some women receive their diagnosis after a pathologist has tested a sample of tissue that has been removed during surgery. Unfortunately, some women are diagnosed with gynaecological sarcoma when the cancer has spread to other parts of the body.
Laparoscopic power morcellation
This technique cuts tissue such as the uterus or fibroids into smaller pieces so they can be removed more easily. However, there is a risk that if a fibroid is an unidentified sarcoma it can be accidentally spread to the abdominal and pelvic cavities. Women over 50, who are post-menopausal and have post-menopausal bleeding, are at an increased risk. The Royal College of Obstetricians and Gynaecologists have published consent advice and patient information for women who are worried about this technique or speak to Sarcoma UK’s Support Line.
Types of diagnostic scans
Uses x-radiation to produce images of the inside of the body.
A scan that uses sound waves to create images from within the body. An ultrasound probe is moved over the part of the body being scanned. Gel is placed on the skin beforehand to help the scan work better.
An internal ultrasound scan; a probe is placed inside the vagina to get a closer look at the area that is being examined.
A procedure used to examine the inside of the uterus (womb). A narrow tube with a telescope at the end called a hysteroscope is inserted through the vagina to take images of the womb.
The Computer Tomography (CT) scan takes a number of x-rays to make a 3D image of an affected area
The Positron Emission Tomography (PET) scan involves an injection of a very small amount of a radioactive drug into the body. The drug helps show how tissues in the body are working. It can help to diagnose and stage a cancer.
Magnetic Resonance Imaging (MRI) uses magnets to create an image of the tissues of the body.
Testing a tissue sample by a pathologist under a microscope to identify disease.
Testing a blood sample to identify infection, disease or to check your general health.
Understanding the diagnosis
A diagnosis of gynae sarcoma should be confirmed by a specialist sarcoma pathologist who will identify the type of sarcoma and the stage and grade of the tumour. Identifying the stage and grade of a cancer means your doctor can advise on the best course of treatment for you. It also describes the cancer in a common language which is useful when your doctor is discussing your case with other doctors or healthcare professionals. The stage of cancer is measured by how much it has grown or spread which can be seen on the results of your tests and scans. The results from a biopsy can tell the grade of the cancer.
- Low-grade means the cancer cells are slow-growing, look quite similar to normal cells, are less aggressive, and are less likely to spread
- Intermediate-grade means the cancer cells are growing slightly faster and look more abnormal
- High-grade means the cancer cells are fast growing, look very abnormal, are more aggressive and are more likely to spread
The staging system used to see how far a gynaecological sarcoma has spread is called the FIGO system. This is different from the staging system of most types of cancer and is specifically used to stage cancers of the cervix, uterus, ovary, vagina and vulva.
- Stage 1a means the cancer is small (less than 5cm) and has not spread to other parts of the body
- Stage 1b means the cancer is larger than 5cm and has not spread to other parts of the body
- Stage 2a means the cancer is of any grade, usually larger than stage one and has spread to other parts of the female reproductive system
- Stage 2b means the cancer has spread to tissues in the pelvis other than the female reproductive system
- Stage 3a means a high grade cancer that has spread to tissues in the abdomen in one site
- Stage 3b means a high grade cancer that had spread to tissues in the abdomen in more than one site
- Stage 3c means the cancer has spread to the lymph nodes
- Stage 4a means a cancer of any grade or size that has spread to the bladder and/or rectum (back passage)
- Stage 4b means a cancer of any grade or size that has spread to a distant part of the body from the original tumour, eg the lungs
Your case will be managed by a team of experts from a wide range of health care professions called a multidisciplinary team (MDT). Your MDT will include your key worker or clinical nurse specialist, surgeon and other healthcare professionals involved in your care. Your MDT will support you throughout your treatment to ensure you get the right treatment as and when you need it.
The National guidelines in England and Wales recommend that a sarcoma MDT is involved at an early stage of treatment for women with gynaecological sarcomas.
If your gynaecological sarcoma is diagnosed before surgery, following investigations and tests, your treatment should be managed jointly by a sarcoma MDT and a gynaecological oncology (cancer) MDT. Surgery for gynaecological sarcoma should ideally be carried out by a surgeon with specialist expertise in gynaecological cancers, working together with sarcoma specialists to ensure you get the right treatment.
If your gynaecological sarcoma is diagnosed after surgery, you should be referred immediately to a sarcoma MDT who will review your case, manage your ongoing care, and decide on the best treatment options available to you in the future.
In Scotland, women with gynaecological sarcomas are treated by the gynaecological cancer specialist team.
In Northern Ireland women with gynaecological sarcomas are treated by the gynaecological cancer team. They will consult with the sarcoma team on any possible gynaecological sarcoma diagnoses.
Sometimes, the gynaecological team or gynaecological oncology team do not refer women with gynaecological sarcomas to a sarcoma MDT. Always check whether your case has been referred to a sarcoma MDT and request that this is done. Patients who are being treated under a private gynaecologist should also be referred to a sarcoma MDT.
Types of treatment
The type of treatment you receive will depend on the stage and grade of your cancer, the type of gynaecological sarcoma you have, and when it is diagnosed (before or after surgery).
In a lot of cases, the first treatment method for gynaecological sarcoma is surgery to remove the female reproductive organs called a hysterectomy. The main type of hysterectomy performed is a total abdominal hysterectomy with bilateral salpingo-oophorectomy - a hysterectomy that involves taking the fallopian tubes (salpingectomy) and the ovaries (oophorectomy).
Some pre-menopausal women with a uterine leiomysarcoma may retain their ovaries if they wish. This operation is known as a total abdominal hysterectomy - an operation to remove the uterus and cervix but not the ovaries and fallopian tubes. You can discuss this option with your doctor.
In younger women who are still having their periods, removal of the ovaries will bring on early menopause and lead to a loss of fertility. Please speak to your doctor prior to surgery to discuss your options for preserving your fertility.
This treatment uses high-energy radiation beams to destroy cancer cells delivered as external beam radiotherapy or occasionally delivered internally with brachytherapy, which involves putting a solid radioactive source close to, or placed into, the tumour. Radiotherapy is not commonly used in the adjuvant setting for uterine sarcoma but it may be used on leiomyosarcomas or endometrial stroma; sarcomas that have extended outside the uterus into the pelvic region. In this case, the aim is to kill off any local cancer cells which remain in the area of the tumour.
This treatment uses anti-cancer drugs to destroy cancer cells. Not all gynaecological sarcomas respond well to this type of treatment and it is not commonly used to treat endometrial stromal sarcoma. It is mostly used to treat high grade leiomyosarcomas and undifferentiated endometrial sarcomas that have spread to other parts of the body. Some chemotherapy drugs used for gynaecological sarcoma (particularly leiomyosarcomas) include doxorubicin, gemcitabine, doxetaxel, trabectidin, darcarbizine and pazopanib. Less frequently, ifosfamide is used to treat undifferentiated endometrial sarcoma.
You will be given more information about the type of treatment most suitable for you by your treatment team.
Many gynaecological sarcomas are stimulated to grow by the female growth hormones oestrogen and progesterone. These sarcomas are called hormone sensitive or hormone positive.
Around half of leiomysarcomas in the uterus are hormone positive and endometrial stromal tumours are almost always hormone positive. Ideally, your tumour should be hormone tested after your initial surgery and the results will either be positive or negative. You can ask your oncologist to confirm whether your tumour has been hormone tested or not.
Hormone treatment is given following surgery to lower the risk of the cancer coming back or to help control advanced cancer.
Hormone therapy or anti-oestrogen therapy, uses types of drugs called aromatase inhibitors or progesterone that stops oestrogen being made in the fatty tissues and can help prevent the tumour growing. This treatment may be used if there is oestrogen present in the tumour. Your treatment team will advise you on whether this is an appropriate treatment for you.
Hormone replacement therapy
If you have previously been on hormone replacement therapy, please speak to your doctor to enquire as to whether this type of treatment is appropriate for you.
Women who are being treated with aromatase inhibitors may have a higher risk of osteoporosis. You may want to talk to your doctor about exploring the use of
bisphosphonate therapy to protect your bone density levels whilst on this type of treatment. Women on this type of treatment should have regular bone density scans to monitor their bone density levels.
You may be offered an opportunity to take part in a study to investigate new diagnosis methods, drugs or treatments for gynaecological sarcomas. Some studies also look at the care and well-being of patients. Your doctor or nurse can give you more information on opportunities for you to take part in a clinical trial or take a look at our Clinical Trials Hub.
Side effects of treatment
You may experience side effects from your treatment. The type of side effects will depend on the type of treatment you have received. Don’t be afraid to ask your clinical nurse specialist if any symptoms you are experiencing are a side effect of your treatment. You clinical nurse specialist can also help if your symptoms are troublesome or persistent.
After treatment, you will have regular follow up appointments for several years. You should receive a follow-up schedule from your sarcoma clinical nurse specialist. The usual practice will include:
• A chance to discuss symptoms
• An examination to look for any signs of the sarcoma returning. This may include an MRI or ultrasound if required after examination
• An x-ray of the pelvis to rule out any cancer in the local region
• A chest x-ray or CT scan to rule out any secondary cancers occurring in the lungs or liver
What if my cancer spreads?
A gynaecological sarcoma can reappear in the same area after the treatment of a previous tumour; this is called a recurrence. A recurrence of sarcoma may be accompanied by cancer in other parts of the body; this is called metastasis or secondary cancer. Some people are diagnosed with sarcoma because their metastases have been discovered before their primary sarcoma tumour. In sarcoma patients, these secondary cancers may appear in the lungs or liver, which is why a chest x-ray or CT scan is performed before follow-up appointments.
Secondary gynaecological sarcomas may also appear in the abdomen. Treatment for secondary cancer may involve surgery, radiotherapy or chemotherapy as appropriate; your treatment will be assessed on an individual basis. Your sarcoma MDT will review the treatments available to you and may suggest a clinical trial.