Ovarian Carcinosarcoma

Ovarian cancer is a type of carcinoma that causes one or more tumours to develop in the ovaries. The ovaries are a pair of small, walnut shaped organs that are responsible for the release of eggs (ovum) during the female reproductive cycle.

Ovarian carcinosarcoma, also known as malignant mixed Müllerian tumours (MMMT) of the ovary or ovarian malignant mixed epithelial mesenchymal tumours, are a very rare and often aggressive subtype of this disease that has both epithelial and stromal components. These tumours have characteristics of two types of cancers: carcinomas (cancers arising from the tissues that line organs) and sarcomas (cancers arising from bones and/or soft tissue).

Ovarian carcinosarcomas are generally diagnosed in women between the ages of 60-80, however, it can affect almost anyone with ovaries – including women, teenagers, transgender men, non-binary individuals, and intersex people – at any age.

Treatment

If an ovarian cancer is detected, it will be staged and graded based on size, metastasis, and how the cancer cells look under the microscope. Staging and grading helps your doctors determine the best treatment for you.

Ovarian cancers can be staged using the Federation of Gynecology and Obstetrics (FIGO) system from stage I to IV:

  • Stage I: cancer cells are confined to one or both ovaries only. This stage is also known as early-stage cancer.
  • Stage II: cancer cells have grown deeper into nearby organs in the pelvis, such as the uterus, fallopian tubes, bladder and/or bowel. This is also known as localised cancer.
  • Stage III: the cancer has become larger and has spread beyond the pelvis into the lining of the abdomen (peritoneum). Lymph nodes are also often affected. This is also known as advanced or metastatic cancer.
  • Stage IV: the cancer has spread to more distant organs, such as the lungs or the liver. This is also known as advanced or metastatic cancer.

Cancers can also be graded based on the rate of growth and how likely they are to spread:

  • Grade I: cancer cells present as slightly abnormal and are usually slow growing. This is also known as a low-grade tumour.
  • Grade II: cancer cells present as abnormal and grow faster than grade-I tumours. This is also known as an intermediate-grade tumour.
  • Grade III: cancer cells present as very abnormal and grow quickly. This is also known as a high-grade tumour.

Once your tumour has been staged and graded, your doctor may recommend genetic testing, which analyses your tumour DNA and can help determine which treatment has the greatest chance of success. In particular, they may look for mutations in the hereditary breast cancer genes (BRCA1 and BRCA2), which can cause an increased risk in developing ovarian cancer. They will then discuss the most appropriate treatment option for you.

Treatment is dependent on several factors, including fertility, type, stage of disease and overall health.

Treatment options for ovarian carcinosarcomas may include:

  • Surgery, potentially including:
    • Hysterectomy.
    • Bilateral salpingo-oophorectomy.
    • Unilateral salpingo-oophorectomy.
    • Lymphadenectomy.
  • Chemotherapy.
  • Radiation therapy.
  • Targeted therapy.
  • Clinical trials.
  • Palliative care.

Ovarian Cancer Treatment and Fertility

Treatment for ovarian cancer may make it difficult to become pregnant. If fertility is important to you, discuss your options with your doctor prior to the commencement of treatment.

Risk factors

While the cause of ovarian cancer remains unknown, the following factors may increase your risk of developing the disease:

  • Having a family history of ovarian, breast, uterine and/or bowel cancer.
  • A mutation in the BRCA 1 and BRCA 2 genes.
  • Having Lynch syndrome.
  • Having certain medical conditions such as endometriosis.
  • Use of hormone replacement therapy (HRT).
  • Having a history of tobacco smoking.
  • Being obese.
  • Reproductive history, including:
    • Women who have never had children.
    • Women who have had assisted reproduction.
    • Women who have had children over the age of 35.
  • Having early puberty.
  • Having late menopause.
  • Having Ashkenazi Jewish ancestry.

Not everyone with these risk factors will develop the disease, and some people who have the disease may have none of these risk factors. See your general practitioner (GP) if you are concerned.

Symptoms

Early-stage ovarian carcinosarcomas may be asymptomatic. As the cancer progresses, some of the following symptoms may appear:

  • Abdominal bloating/swelling.
  • Abdominal/pelvic pain and/or pressure.
  • Changes in appetite, such as feeling full quickly or not feeling hungry.
  • Urinary changes, such as changes in frequency and urgency.
  • Changes in bowel habits, such as constipation and diarrhoea.
  • Unexplained weight loss or weight gain.
  • Unexplained fatigue.
  • Digestive problems.
  • Changes in menstrual periods, such as irregular periods, unusual vaginal bleeding, or vaginal bleeding post menopause.
  • Pain during sex.

Not everyone with the symptoms above will have cancer, but see your general practitioner (GP) if you are concerned.

Diagnosis

If your doctor suspects you have ovarian cancer, they may order the following tests to confirm the diagnosis and refer you to a specialist for treatment:

  • Pelvic examination.
  • Imaging tests, potentially including:
    • Pelvic ultrasound.
    • Transvaginal ultrasound.
    • CT (computed tomography) scan.
    • PET (positron emission tomography) scan.
  • Blood tests.
  • Biopsy.

References

Keep up with Rare Cancers Australia

Inside Rare is a monthly newsletter that shares the latest news, events and stories connecting the rare community.