Ovarian sex cord-stromal tumors

Sex cord-stromal tumors are an unusual group of ovarian tumors that come from the hormone-producing tissue of the ovary rather than its surface. Because many of them make hormones, they often announce themselves through hormonal symptoms — and they are tracked with hormone-related markers, not CA125. A normal CA125 says little about this group.

What it is and how common it is

The ovary contains supporting “stromal” tissue and specialized “sex cord” cells that normally produce hormones like estrogen and, in smaller amounts, androgens. Tumors that arise from these cells make up a minority of ovarian tumors. The group includes:

• Granulosa cell tumors — the most common malignant type, occurring in an adult form (usually in middle-aged or older women) and a rarer juvenile form (in girls and young women)
• Sertoli-Leydig cell tumors — which can produce male hormones
• Thecomas and fibromas — which are usually benign

A hallmark of many sex cord-stromal tumors is that they are hormonally active. Adult granulosa cell tumors, for example, are often associated with a specific gene change (FOXL2), and they can behave indolently but recur many years — even decades — after the original diagnosis, which makes long-term follow-up important.

How they behave on a CA125 test

CA125 is usually not helpful for sex cord-stromal tumors. Instead, these tumors are best tracked with markers that reflect the hormone-producing cells they come from:

• Inhibin (A and B) — often raised in granulosa cell tumors
• Anti-Müllerian hormone (AMH) — another marker associated with granulosa cell tumors
• Estradiol — reflecting estrogen production by the tumor
• In androgen-producing tumors, testosterone and related hormones may be raised

Because these tumors can recur late, these markers are also used for long-term monitoring. Relying on CA125 alone would risk missing both the initial diagnosis and a later recurrence.

Symptoms and how they’re diagnosed

What makes sex cord-stromal tumors distinctive is that hormonal effects are often the first clue — sometimes before any mass is noticed:

• Estrogen effects: abnormal uterine bleeding, bleeding after menopause, or, in young girls, signs of early (precocious) puberty
• Androgen effects: irregular or absent periods, or signs of virilization such as excess hair growth or voice changes (more typical of Sertoli-Leydig tumors)
• Mass effects: pelvic pressure, pain, or abdominal swelling
• Some granulosa cell tumors can bleed, occasionally causing sudden pain

Because estrogen-producing tumors can also thicken the uterine lining, abnormal bleeding may prompt evaluation of the endometrium in addition to the ovaries. Diagnosis combines imaging, hormone and marker testing (inhibin, AMH, estradiol), and — as with all ovarian tumors — pathology after surgery.

Treatment and outlook

Surgery is the mainstay of treatment, both to remove the tumor and to confirm the diagnosis. Because some patients are young and the tumors are often found early and on one ovary, fertility-sparing surgery may be possible in appropriate cases. Chemotherapy or hormonal therapy may be used for more advanced or recurrent disease, guided by a specialist.

Many sex cord-stromal tumors, especially adult granulosa cell tumors caught early, have a favorable outlook. The important caveat is their tendency to recur late, which is why long-term monitoring with the relevant markers is part of care even after successful treatment. Prognosis depends on the type, stage, and how completely the tumor is removed.

Questions to ask your care team

• Which specific type of sex cord-stromal tumor do I have?
• Which markers (inhibin, AMH, estradiol) will be used to follow me over time?
• Given the risk of late recurrence, what does long-term monitoring look like?
• Is fertility-sparing surgery an option in my case?
• Should my uterine lining be evaluated because of the hormone effects?

Why this matters to us

Sex cord-stromal tumors show just how differently ovarian cancers can behave — and how important it is to use the right test for the right tumor. The Cure Ovarian Cancer Foundation funds research across the full range of ovarian cancers and supports the families living with them for the long haul. If this guide helped you, please consider donating so we can be there for the next person, too.

This page is for education and awareness and is not medical advice. Please talk with your own care team about your diagnosis, monitoring, and treatment.