Ovarian neuroendocrine tumours (NETs), also known as ovarian carcinoid tumours, are a very rare type of neuroendocrine tumoura tissue mass that forms from groups of unhealthy cells that develops 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.
Neuroendocrine cancers are a complex group of tumours that develop in the neuroendocrine system, which is responsible for regulating important bodily functions such as heart rate, bloodthe red bodily fluid that transports oxygen and other nutrients around the body pressure and metabolism. They most commonly develop in the gastro-intestinal tract, pancreasa long, flat organ that sits between the stomach and the spine that plays a key role in digestion and blood sugar regulation, and the lungs; however, they can develop anywhere in the body. These tumours develop from neuroendocrine cellsthe basic structural and functional unit of all living things, which are responsible for receiving signals from the nervous system and producing hormones and peptides (small proteins) in response.
Ovarian NETs are most common in post-menopausal women who are over 50 years old, however it can affect anyone with ovaries – including pre-menopausal women, teenagers, transgender men, non-binary individuals, and intersex people – at any age.
Treatment
If an ovarian NET is detected, it will be staged and graded based on size, metastasiswhen the cancer has spread to other parts of the body, also known as mets, and how the cancera disease where abnormal cells split without control and spread to other nearby body tissue and/or organs cells look under the microscope. Stagingthe process of determining how big the cancer is, where it started and if it has spread to other areas and grading helps your doctors determine the best treatment for you.
FIGO Staging System
Gynaecological cancers, such as ovarian NETs, can be staged using the Federation of Gynaecology 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 bowelportion of the digestive system that digests food (small bowel) and absorbs salts and water (large bowel); also called intestines. 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 (peritoneumthe membrane that lines the abdominal cavity). 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 advancedat a late stage, far along or metastatic cancer.
TNM Staging System
The TNM system can also be used to classify an ovarian NET. The TNM system is comprised of:
- T (tumour) indicates the size and depth of the tumour.
- N (nodea small lump or mass of tissue in your body) indicates whether the cancer has spread to nearby lymph nodessmall bean-shaped structures that filters harmful substances from lymph fluid.
- M (metastasis) indicates whether the cancer has spread to other parts of the body.
This system can also be used in combination with a numerical value, from stage 0-IV:
- Stage 0: this stage describes cancer cells in the place of origin (or ‘in situ’) that have not spread to nearby tissuea group of cells that work together to perform a function.
- Stage I: cancer cells have begun to spread to nearby tissue. It is not deeply embedded into nearby tissue and had not spread to lymph nodes. This stage is also known as early-stage cancer.
- Stage II: cancer cells have grown deeper into nearby tissue. Lymph nodes may or may not be affected. This is also known as localisedaffecting only one area of body cancer.
- Stage III: the cancer has become larger and has grown deeper into nearby tissue. Lymph nodes are generally affected at this stage. This is also known as localised cancer.
- Stage IV: the cancer has spread to other tissues and organs in the body. 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.
- Gradea description of how abnormal cancer cells and tissue look under a microscope when compared to healthy cells 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 testinga procedure that analyses DNA to identify changes in genes, chromosomes and proteins, which can be used to analyse tumour DNA to help determine which treatment has the greatest chance of success, which analyses your tumour DNA and can help determine which treatment has the greatest chance of success. They will then discuss the most appropriate treatment option for you.
Treatment Options
Treatment is dependent on several factors, including location, stage of disease and overall health.
Treatment options for ovarian NETs may include:
- Surgerytreatment involving removal of cancerous tissue and/or tumours and a margin of healthy tissue around it to reduce recurrence, potentially including:
-
- Hysterectomycomplete or partial removal of the uterus.
-
- Bilateral salpingo-oophorectomyremoval of both ovaries and fallopian tubes.
-
- Unilateral salpingo-oophorectomyremoval of one ovary and fallopian tube.
-
- Anterior pelvic exenterationremoval of reproductive organs (uterus, cervix, ovaries, fallopian tubes), part or all of the vagina, pelvic lymph nodes, urethra, lower portion of the ureters and the bladder.
-
- Posterior pelvic exenterationremoval of reproductive organs (uterus, cervix, ovaries, fallopian tubes), part or all of the vagina, pelvic lymph nodes, lower portion of the bowel and the rectum.
-
- Total pelvic exenterationremoval of reproductive organs (uterus, cervix, ovaries, fallopian tubes), part or all of the vagina, pelvic lymph nodes, lower portion of the bowel, rectum, urethra, lower portion of ureters and the bladder.
- Somatostatin analogues (SSAs)medication used to inhibit excessive hormone production .
- Chemotherapya cancer treatment that uses drugs to kill or slow the growth of cancer cells, while minimising damage to healthy cells.
- Radiation therapya treatment that uses controlled doses of radiation to damage or kill cancer cells, potentially including brachytherapya type of internal radiation therapy where radioactive material is placed inside or near a tumor to deliver targeted radiation.
- Targeted therapymedication that targets specific molecular features of cancer cells.
- Clinical trialsresearch studies performed to test new treatments, tests or procedures and evaluate their effectiveness on various diseases.
- Palliative carea variety of practices and exercises used to provide pain relief and improve quality of life without curing the disease.
Ovarian Neuroendocrine Tumour Treatment and Fertility
Treatment for ovarian NETs may make it difficult to become pregnant. If fertility is important to you, discuss your options with your doctor and a fertility specialist prior to the commencement of treatment.
Risk factors
Because of how rare ovarian NETs are, there has been little research done into the riskthe possibility that something bad will happen factors of this disease.
Symptoms
Some patients with ovarian NETs will appear asymptomatic in the early stages of disease. As the tumour 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 constipationa condition where a person has difficulty passing faeces/stools and diarrhoeafrequent discharge of watery or loose stools from the body.
- Unexplained weight loss or weight gain.
- Unexplained fatiguea state of extreme tiredness or exhaustion, can be physical or mental.
- Indigestion and/or heartburn.
- Changes in menstrual periods, such as irregular periods, unusual vaginal bleeding, or vaginal bleeding post menopause.
- Pain during intercourse.
- Bleeding after intercourse.
- Carcinoid syndrome (rare), which carries its own set of symptoms:
-
- Facial flushing (usually red or purple in the face, neck, and/or upper chest).
-
- Diarrhoea.
-
- Wheezing.
-
- Abdominal pain.
-
- Carcinoid heart disease (plaques on the heart muscle caused by excess hormonea chemical substance produced by glands in the endocrine system that regulates various functions in the body production).
-
- Faecal urgency.
-
- Fatigue.
-
- Skin changes, such as red or purple spots on the face, neck, and/or upper chest.
- Paraneoplastic syndromesa group of rare disorders that occur when the immune system has a reaction to a cancerous tumour within the body (rare).
- Hypercalcaemiaexcess levels of calcium in the blood (rare).
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 an ovarian NET, they may order the following tests to confirm the diagnosisthe process of identifying a disease based on signs and symptoms, patient history and medical test results and refer you to a specialist for treatment:
- Physical examinationan examination of your current symptoms, affected area(s) and overall medical history.
- Pelvic examinationa physical exam of the external and internal female pelvic organs.
- Imagingtests that create detailed images of areas inside the body tests, potentially including:
-
- Pelvic ultrasounda type of medical imaging that uses sound waves to create detailed images of the organs and structures within the pelvis, including the uterus, ovaries, and surrounding tissues.
-
- Transvaginal ultrasounda type of pelvic ultrasound that involves inserting a device (known as a transducer) into the vagina to produce sound waves and create images of internal female reproductive organs in greater detail.
-
- MRI (magnetic resonance imaging)a type of medical imaging that uses radiowaves, a strong magnet and computer technology to create detailed images of the body.
-
- CT (computed tomography) scana type of medical imaging that uses x-rays and computer technology to create detailed images of the body.
-
- PET (positron emission tomography) scana type of medical imaging that uses radioactive tracers to create detailed images of the body.
- Blood teststesting done to measure the levels of certain substances in the blood.
- Exploratory surgeryan exploratory surgical procedure used for conditions that cannot be confirmed by scans and tests alone, such as a diagnostic laparoscopya minimally invasive surgical procedure that uses small incisions to access the body for procedures, often used for conditions that cannot be confirmed by scans and tests alone.
- Biopsyremoval of a section of tissue to analyse for cancer cells.