Ovarian, fallopian tube and peritoneal cancer

• About 90 out of 100 ovarian cancers. Usually after 40 years of age.
• High-grade serous is the most common; others are low-grade serous, endometrioid, clear cell and mucinous.
• About 1 in 5 is linked to an inherited BRCA mutation.

• Starts in the fallopian tube, usually at its far end near the ovary.
• Most high-grade serous cancers are now thought to begin here, even when the ovary looks like the main site.
• Tests and treatment are the same as for epithelial ovarian cancer. BRCA testing is advised for every patient.

• Starts in the lining of the tummy (peritoneum), with the ovaries normal or only lightly involved.
• Usually causes fluid in the tummy and is found at stage 3 or 4.
• Can occur even after the ovaries have been removed. Treated in the same way as ovarian cancer.

• Occur in girls and young women.
• Usually respond very well to surgery and chemotherapy (BEP); most are cured, and fertility can often be preserved.

Rare tumours such as granulosa cell tumour, which can make hormones and cause irregular bleeding. Usually slow growing; surgery is the main treatment.

Show how far the cancer has spread in the tummy, chest and lymph nodes, and help the surgeon plan.

• CA-125: raised in most epithelial cancers; used to follow treatment.
• CEA and CA 19-9: to make sure the cancer has not started in the bowel or stomach.
• In young women: AFP, beta-hCG and LDH for germ cell tumours.

A needle biopsy of the tumour or omentum, or fluid testing, confirms the cancer when chemotherapy is given first. Otherwise the diagnosis is made at surgery. A diagnostic laparoscopy helps decide whether complete removal is possible.

• BRCA1/2 (inherited) test for every woman with epithelial ovarian, fallopian tube or primary peritoneal cancer.
• Tumour BRCA and HRD test: shows whether PARP inhibitor tablets (olaparib, niraparib) will help.
• If a BRCA mutation is found, sisters, daughters and other relatives can be tested. Hereditary cancers

Before starting chemotherapy, immunotherapy or targeted therapy, these tests check that you are fit for treatment, and give a starting point to compare with later.

Before immunotherapy, also:

Sometimes needed, depending on your treatment:

• Pregnancy test for women who could be pregnant.
• Calcium and magnesium: for example before cisplatin or bone-strengthening injections.
• Dental check-up before bone-strengthening injections (zoledronic acid or denosumab), as they can rarely affect the jaw bone.
• Hearing test before cisplatin, in some patients.
• Clotting tests (PT/INR) before a biopsy or chemoport.

Your doctor will choose the tests that apply to you. Some of them are repeated during treatment.

• The scans and laparoscopy decide whether surgery or chemotherapy comes first.
• Surgery is most successful when all visible cancer can be removed; planning it properly matters more than doing it quickly.
• BRCA and HRD results decide the maintenance tablets after chemotherapy.
• Waiting a week or two for these reports does not make the cancer worse.

Limited to one or both ovaries or fallopian tubes. The aim is cure.

Spread to other organs in the pelvis, such as the womb or bladder surface. The aim is cure.

Spread to the lining of the tummy (peritoneum) outside the pelvis, or to lymph nodes. This is the commonest stage at diagnosis, and primary peritoneal cancer is always stage 3 or 4. Treatment aims for long-term control, and cure in some.

Spread beyond the tummy: fluid around the lungs, inside the liver, or other organs.

1. Staging surgery: removal of the womb, both ovaries and tubes, omentum, and samples from the tummy and lymph nodes.
2. Young women with early, low-risk cancer: fertility-sparing surgery (keeping the womb and one ovary) may be possible.
3. Chemotherapy (carboplatin with paclitaxel, 3 to 6 cycles) for most, except very early low-risk cancers.

Side effects: surgery · chemotherapy

1. Surgery and chemotherapy, in one of two orders:
   • Surgery first, then 6 cycles of chemotherapy, or
   • 3 cycles of chemotherapy first (neoadjuvant), then surgery (interval surgery), then 3 more cycles. HIPEC (heated chemotherapy in the tummy during surgery) is used in some centres.
2. The goal of surgery is to remove all visible cancer.
3. Chemotherapy: carboplatin with paclitaxel every 3 weeks. Bevacizumab may be added.
4. Maintenance tablets after chemotherapy, chosen by the BRCA and HRD results:
   • BRCA mutation: olaparib for 2 years (or niraparib).
   • HRD positive: olaparib with bevacizumab, or niraparib.
   • HRD negative: niraparib, or bevacizumab alone.

Side effects: chemotherapy · PARP inhibitors · bevacizumab · surgery

1. Depends on how long after the last platinum chemotherapy it returns:
   • More than 6 months (platinum-sensitive): platinum chemotherapy again, often with bevacizumab, then a PARP inhibitor if not used before. Surgery helps some.
   • Less than 6 months (platinum-resistant): weekly paclitaxel, liposomal doxorubicin or other chemotherapy, often with bevacizumab.
2. Mirvetuximab soravtansine for cancers with high FR-alpha (folate receptor) on testing.

Side effects: chemotherapy · bevacizumab and mirvetuximab

1. Fertility-sparing surgery in most young women.
2. BEP chemotherapy (bleomycin, etoposide, cisplatin) for most stages.
3. Cure rates are very high, even when the cancer has spread.

Side effects: chemotherapy

• Nausea and vomiting: well controlled with medicines given before and after chemotherapy.
• Low blood counts and infection risk: fever of 38°C or more needs urgent care.
• Tiredness, mouth sores, loose stools or constipation.
• Hair loss with paclitaxel; it grows back.
• Tingling and numbness in hands and feet with paclitaxel.
• Allergic reactions during the drip, mostly with paclitaxel early on and with carboplatin after many cycles. Medicines are given beforehand.

More about chemotherapy · Side effects of your regimen

• Olaparib, niraparib: tiredness, nausea, low haemoglobin and platelets (blood tests every month at first), and high blood pressure with niraparib.
• Rarely, a blood disorder (MDS or leukaemia) after long use.

More about targeted therapy · Side effects of your regimen

• Bevacizumab: high blood pressure, protein in urine, nosebleeds; rarely a hole in the bowel (severe tummy pain needs urgent care).
• Mirvetuximab: blurred vision and dry eyes. Eye drops are used and eyes are checked regularly.

• A major operation; recovery takes several weeks. A temporary stoma is sometimes needed.
• If the ovaries are removed before menopause: hot flushes and other menopause symptoms. Ask whether hormone replacement is safe for you.

Vomiting with a swollen tummy and no stools or wind (possible bowel blockage), severe tummy pain, fever of 38°C or more, breathlessness, or leg swelling need urgent care.

When to seek help urgently