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Ovacome is a national charity providing support to anyone affected by ovarian cancer. We give information about symptoms, diagnosis, treatments and research. Ovacome runs a telephone and email support line and works to raise awareness and give a voice to all those affected by ovarian cancer.

This booklet is part of a series giving clinical information about ovarian cancer.  It explains what clear cell carcinoma of the ovary is and how it is treated. There is a glossary with this booklet, to explain words you may not be familiar with.

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What is clear cell carcinoma of the ovary?

Clear cell carcinoma of the ovary is an uncommon type of epithelial ovarian cancer.  It accounts for five to 10 per cent (five to 10 in a hundred) of patients with ovarian cancer in the western world, but 25 per cent (one in four) in East Asia (China, Japan, Korea, Mongolia).

It is likely that the cause of clear cell is different from the most common type of ovarian cancer, high grade serous epithelial ovarian cancer. 

It may develop from other ovarian tumours called adenofibromas.  A family history of clear cell ovarian cancer may also increase the risk and there is an association with Lynch syndrome which is an inherited condition that increases the risk of several cancers. 

Clear cell ovarian cancer may also be more common in people with a history of endometriosis.

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Diagnosis

The symptoms of clear cell ovarian cancer are often experienced at an earlier stage than other types of ovarian cancer.

Those affected may notice a lump in their abdomen or experience pain or discomfort.  There is also an increased risk of developing deep vein thrombosis and pulmonary embolism (a blood clot in the lungs) which can be the first sign of the cancer.

The tumour marker CA125, which is measured in a blood test and used in the diagnosis of ovarian cancer, is not always raised in clear cell cancers so it may be less reliable.

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How is it treated?

The first treatment is the same as for other more common forms of ovarian cancer.  This means surgery; a total abdominal hysterectomy and bilateral salpingo-oophorectomy and omentectomy. This is the removal of the womb, cervix, ovaries, fallopian tubes, and omentum, a fatty sheet of tissue that covers the abdominal organs.  This operation is sometimes referred to as a TAHBSO.

You can get more information about this operation from Ovacome booklet 15 Surgery for ovarian cancer.

The lymph glands in the pelvis and the back of the abdomen may also be removed.  They can show the stage of the cancer, meaning how far it has spread. Fluid from the abdomen will be tested for any cancer cells.  A pathologist will examine the tissue and fluid removed to get more information about how advanced the cancer is.

You can get more information about staging from Ovacome booklet 3 The stages of ovarian cancer.

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Fertility sparing surgery

If the cancer is at stage 1A, which means it is within one ovary or fallopian tube and has not spread any further, it may be possible to have fertility sparing surgery.  This is when the ovary and fallopian tube that are affected are removed, with the womb and other ovary remaining in place.  This means a pregnancy may still be possible.

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Chemotherapy

After surgery you are likely to be offered chemotherapy using two drugs, carboplatin and paclitaxel (Taxol). 

Where all the visible cancer has been removed, the aim of chemotherapy is to reduce the risk of the cancer coming back.  When the tumour is more advanced and cannot be completely removed, the aim is to increase the time until the cancer returns and further treatment is needed.

Clear cell tumours respond less fully to chemotherapy than other ovarian cancers, so the benefit from chemotherapy may be less. 

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What are the side effects of chemotherapy?

If you are recommended to receive the two chemotherapy drugs carboplatin and paclitaxel (Taxol) these will be given as an intravenous infusion (through a drip into a vein) once every three weeks for up to six months.

You may experience side effects from the chemotherapy treatment.  These might include feeling tired, nausea (feeling sick), hair loss and tingling in your fingertips and toes.  Occasionally, you might get pain in your muscles and joints.  You will normally take three to six weeks to recover from this.

You can find more information about chemotherapy and its side effects from Ovacome booklet 20 Chemotherapy.

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What happens if the cancer comes back?

If your cancer comes back or starts growing again after your initial treatment, the standard treatment for recurrence is to use the same chemotherapy as for other ovarian tumours.

However, because clear cell cancers respond less to chemotherapy there are a number of studies looking at different types of drug treatment to see if they are more effective.

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Current research

A trial that studied a different treatment to carboplatin and paclitaxel (Taxol) did not find any advantage so carboplatin and paclitaxel (Taxol) remain the standard treatment.

However, there is research which suggests that for people with clear cell cancer that is confined to the ovary (stage IA, IB and IC1) and where there has been an assessment of the pelvic and para-aortic lymph nodes, chemotherapy may be avoided.  You will need to discuss the risks and benefits for your individual situation with your oncologist.

There is some research to suggest that radiation therapy (radiotherapy) is effective at protecting against recurrence.  It also suggests that intra-peritoneal chemotherapy (where it is delivered directly into the abdomen) may also be effective.  This has not been accepted into routine clinical practice but you could discuss it with your consultant.

A clinical trial is looking at nintedanib, an agent that targets tumour blood vessels.  Another study is trialling a new immunotherapy drug pembrolizumab, and a further study is looking at an ATR inhibitor which blocks clear cell cancer cells’ DNA damage repair systems.  We do not have the results of these studies yet.

If you are interested in taking part in a trial, discuss this with your oncologist. As clear cell cancers are rare, the trials are only open in a few centres but you may be able to be referred to one.

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What happens after treatment finishes

After your treatment, you will need to be carefully followed up by your specialist either in the gynaecology clinic or in the oncology clinic.

Your CA125 level may be checked using a blood test.  If it has gone up or if you are experiencing new symptoms you may be offered a scan.

You will normally be seen every three to four months during the first two to three years, and if there are no changes in your health, every six to 12 months after that.  Follow up usually goes on for up to five years after your treatment, but systems vary. 

Then, if you are feeling well and have no problems it is likely you will have no more follow up care.

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If you would like more information on the sources and references for this fact sheet, please call us on 0800 008 7054. 

If you would like to discuss anything about ovarian cancer, please phone our supportline on 0800 008 7054 Monday to Friday between 10am and 5pm.

You can also visit our website at www.ovacome.org.uk.

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Written by Dr Nicholas Reed,

Beatson Oncology Centre, Gartnavel General Hospital, Glasgow

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Disclaimer 

Ovacome fact sheets provide information and support.  We make every effort to ensure the accuracy and reliability of the information at the time of printing.  The information we give is not a substitute for professional medical care.  If you suspect you have cancer you should consult your doctor as quickly as possible.  Ovacome cannot accept liability for any inaccuracy in linked sources.

v.2.3

Date last updated September 2020

Date for review September 2022

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Glossary

 

Bilateral salpingo-oophorectomy

Removal of ovaries and fallopian tubes.

 

CA125

A protein found in the blood. The amount of CA125 will be higher in those with ovarian cancer.

 

Chemotherapy

Treating cancer with medication, such as cytotoxic drugs (that kill cancer cells).

 

Epithelial ovarian cancer

Ovarian cancer developing from cells covering or lining the ovaries. The most common type of ovarian cancer.

 

Intravenous infusion

How medication is given into the vein over a period of time using a drip.     

 

Lymph glands

Small pearl-like glands connected to the lymph system. They act as filters to bacteria and cancer cells.

 

Omentum

A sheet of fat in the abdomen which protects the intestines.

 

Para-aortic lymph nodes 

A group of lymph nodes that lie near the aorta in front of the lumbar vertebrae.    

 

Pathologist

A specialist who examines parts of the body affected by disease.

 

Post-operative treatment

Treatment after surgery. This is usually chemotherapy or drug therapy.

 

Prognosis

An assessment of how the disease is expected to behave.

 

Stage

Classification given to a cancer depending on how far it has spread. This can be done using scans, blood tests or surgery.

 

Total abdominal hysterectomy

Surgically removing the uterus and cervix.

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Did you find this fact sheet helpful? We welcome your feedback. If you have any comments or suggestions, please email [email protected] or call 0207 299 6653.

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