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 other cells develop.

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

Types of sex cord stromal tumours

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

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. They 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. Tumours which don’t produce hormones are called non functioning tumours.

Symptoms of sex cord stromal tumours

The symptoms of SCSTs might include:

changes to your periods such as irregular or heavy bleeding
vaginal bleeding after your menopause
pain in your tummy (abdomen) or lower part of your abdomen (pelvis) that doesn't go away
a lump in your abdomen or pelvis
bloating or an increase in the size of your abdomen
constipation
passing urine more often than usual

Diagnosing sex cord stromal tumours

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. This includes inhibin, CA125 and anti mullerian hormone (AMH)
CT scan
Chest x-ray
MRI scan
PET scan

Grading and staging of sex cord stromal tumours

The grade and stage of your cancer is very important. They help your specialist to decide what treatment you need.

Grade

The grade describes how the cells look under a microscope. The less developed the cells look, the higher the grade.

There are 3 different grades – grade 1, grade 2 and grade 3. Generally, grade 1 cancers are the slowest growing and least likely to spread. Higher grade cancers grow more quickly.

Your doctor might also describe your cancer as low grade (grade 1 cancers) or high grade (grade 2 and 3 cancers).

Stage

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:

stage 1 means the cancer is only in the ovary (or both ovaries)
stage 2 means the cancer has spread into the fallopian tube, womb, or elsewhere in the area circled by your hip bones (your pelvis)
stage 3 means the cancer has spread to the lymph nodes or to the tissues lining the abdomen (called the peritoneum)
stage 4 means the cancer has spread to another body organ some distance away, for example the lungs or liver

Treatment for sex cord stromal tumours

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 people 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. For many women it is the only treatment they need.

If you’ve had the menopause

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

Diagram showing parts of the body removed with a hysterectomy

If you’re still having periods

Your doctor will be aware that you might want to have children in the future. If you have an early stage tumour in only one ovary the gynaecologist may only removes the affected ovary. They leave your other ovary and womb intact.

The surgery

During the operation, the gynaecologist examines the inside of your abdomen for signs of cancer. They may take biopsies and send them to the laboratory 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 people, 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

Radiotherapy

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

Follow up

After you've finished your treatment, your healthcare team will see you regularly. They ask how you are feeling, whether you have had any symptoms or side effects, and if you are worried about anything. You might also have tests at some visits.

Tests may include:

a physical examination
blood tests
ultrasound scan
chest x-ray
CT scan

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.

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.

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 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.