Sex cord stromal tumours

There are different types of ovarian sex cord stromal tumours (SCSTs). These can be non cancerous (benign) or cancerous (malignant). Granulosa cell tumours are the most common type.

Treatment depends on several factors including:

your age
the type of tumour you have
how advanced it is (the stage)

Most women are diagnosed at an early stage and treatment usually works well.

What are sex cord stromal tumours?

Sex cord stromal tumours (SCSTs) are rare tumours of the ovary. They start in the stroma or the sex cords. The stroma and the sex cords are tissues that support the ovary. And from which different cells develop.

SCSTs are rare. Out of 100 women with ovarian cancer, around 5 will have a SCST (around 5%).

We don’t know what causes this type of cancer. But we know most cancers are caused by a number of different factors working together.

Types of sex cord stromal tumours

There are 3 main groups of SCSTs:

pure stromal tumours including fibromas and thecomas
pure sex cord tumours including adult and juvenile granulosa cell tumours
mixed sex cord stromal tumours including sertoli–leydig cell tumours

Most pure stromal tumours are non cancerous (benign). Sertoli-leydig tumours can be either benign or cancerous.

The most common type of SCST are granulosa cell tumours which are cancerous (malignant).

Granulosa cell tumours of the ovary

There are 2 types of granulosa cell tumours:

adult granulosa cell tumours are the most common type and are diagnosed in middle age and older women
juvenile granulosa cell tumours are rarer and usually develop before puberty

Granulosa cell tumours often produce hormones and are called functioning tumours. They make the female hormone oestrogen. This can cause symptoms such as abnormal vaginal bleeding or breast tenderness. Tumours which don’t produce hormones are called non functioning tumours.

Symptoms

The symptoms of sex cord stromal tumours might include:

pain in your tummy (abdomen) or lower part of your abdomen that doesn't go away
bloating or an increase in the size of your abdomen
constipation
passing urine more often than usual

Functioning tumours that produce hormones can cause hormone related symptoms including:

changes to your periods such as irregular or heavy bleeding
post menopausal vaginal bleeding
breast tenderness or changes

Diagnosing

Doctors use various tests to diagnose sex cord stromal tumours. These might include:

an ultrasound
blood tests – SCSTs often produce chemicals or hormones (tumour markers) that doctors can measure in the blood, including inhibin, CA125 and anti mullerian hormone (AMH)
CT scan
Chest x-ray
PET scan

Staging

The stage of a cancer tells you how far it has grown. For sex cord stromal tumours, doctors use the same staging system that they use for other types of ovarian cancer. There are 4 stages, from 1 to 4.

Treatment

The type of treatment you have will depend on:

your age and whether you have been through the menopause
whether you want to have children
the stage and type of your tumour

You have surgery to remove the cancer. For some tumours, this is the only treatment you need.

Some women need chemotherapy after surgery. You might have chemotherapy if you have:

juvenile granulosa cell tumour and your cancer is stage 1C or greater
adult granulosa cell tumour and your cancer is stage 1C2 or greater
a sertoli–leydig cell tumour and the cells look very abnormal (poorly differentiated) under the microscope

Surgery

Surgery is the main treatment for this type of ovarian cancer. And for many women it is the only treatment you need.

If you’ve had the menopause

The gynaecologist will remove your ovaries, fallopian tubes and womb. Doctors call this operation a bilateral salpingo oophorectomy (pronounced sal-pin-go-oo-for-eck-tom-ee). And total abdominal hysterectomy.

If you’re still having periods

Your doctor will be aware that you might want to have children in the future. When possible, the gynaecologist (gynaecological oncologist) removes the affected ovary. But leaves your other ovary and womb intact.

They might do this if you have an early stage tumour in only one ovary. And you haven't yet had all the children you want to.

The surgery

During the operation, the gynaecologist examines the inside of your abdomen. And your abdominal organs for signs of cancer. They may take biopsies and send them to the lab to look for cancer cells.

Your gynaecologist also washes out the inside of your abdomen. They then send the fluid to be checked for cancer cells. This is to check that the cancer hasn't spread.

After surgery

Your doctor will talk to you about whether you need chemotherapy after surgery. This depends on several factors. For many women, surgery is the only treatment they need.

The doctor monitors you after surgery. This is to check for signs of the cancer coming back. You might have further surgery if your cancer comes back.

Chemotherapy

You might have chemotherapy:

after surgery – this is called adjuvant chemotherapy
if your cancer has spread (advanced cancer)
if your cancer comes back and you can’t have surgery to remove it

Generally, you have chemotherapy as a combination of different drugs. Doctors use various combinations including:

BEP which stands for bleomycin, etoposide and cisplatin
EP which stands for etoposide and cisplatin – you have this instead of BEP if you are older than 40
carboplatin and paclitaxel

You usually have chemotherapy as several cycles of treatment. Each cycle lasts 3 weeks. You have several days of chemotherapy at the beginning of the cycle and then a break until the start of the next cycle.

All the drugs go into your bloodstream through a vein in your arm (intravenously) or through a central line.

Radiotherapy

You might have radiotherapy to treat the cancer if it comes back. It isn’t usually used as a first treatment.

After treatment

After you've finished your treatment, your specialist will see you regularly. They will examine you and ask you how you are feeling. How often they see you will depend on what tumour you had and your treatment.

You usually have blood tests to check for any changes in the level of the tumour markers. You might have an ultrasound or CT scan from time to time, depending on your situation.

You have tests and see the doctor every few months to begin with. The monitoring gradually becomes less frequent but you carry on having tests every 6 months for many years. This is because there is a chance of the cancer coming back for a long time after treatment.

Coping

Coping with a diagnosis of cancer can be difficult, both practically and emotionally. It can be especially difficult if you have a rare cancer. Being well informed about your cancer and its treatment can help you make decisions and cope with what happens.

Talking to other people who have the same thing can help. But it can be hard to find people who have had a rare type of cancer.

Cancer Research UK’s discussion forum, Cancer Chat, is a place for anyone affected by cancer. You can share experiences, stories and information with other people who know what you are going through.

Or contact the Rare Cancer Alliance who offer support and information to people who have rare cancers.

Research and clinical trials

There may be fewer clinical trials for rare types of cancer than for more common types.

It is hard to organise and run trials for rare cancers. Getting enough patients is critical to the success of a trial. The results won't be strong enough to prove that one type of treatment is better than another if the trial is too small.

The International Rare Cancers Initiative (IRCI) aims to develop more research into new treatments for rare cancers. They are designing trials that involve several countries so that more people will be available to enter trials.