Severe pelvic pain had a sinister cause

An otherwise healthy woman presents with abdominal swelling and pain, Dr Tillmann Jacobi explains

A 42-year-old woman presented with a painful swelling in her lower abdominal region that had developed over the last four weeks. She was usually fit and well and was on no regular medication. She was up to date with her smears, which had all been normal. She felt tired recently but attributed this to increased workload.

On examination I felt a palpable mass in her lower left abdomen without any lymph nodes and an internal examination appeared to be normal.

This woman was found to have a poorly differentiated ovarian epithelial carcinoma of such a size that surgical removal was impossible.

Risk factors
The lifetime risk for ovarian cancer is almost 2 per cent for any woman in the UK.

It affects almost 20 in 100,000 women. The incidence has significantly increased over the last 30 years. This could be partially due to the changing age structure in society, because the majority (85 per cent) of cases occur after the menopause.
 
Ovarian cancer can have a variety of different histologies. The malignant forms occur most commonly in women over 50, whereas the various forms of low malignancy (probably up to about 25 per cent of cases) typically affects younger women around the age of 40.

Germ cell tumours such as dysgerminomas and teratomas are found in younger ages and can have a good prognosis.

Some rare forms (embryonal carcinoma, choriocarcinoma) typically arise in teenagers or even in children.

Ovarian cancer is directly linked to the total number of menstrual cycles in a woman’s life. This is why an early menarche and a late menopause or being nulliparous increases the risk. In addition, hormonal medication can either increase the risk or lower it.

Other risk factors are a personal or family history of ovarian and breast cancer, and possibly exposure to asbestos.

Diagnosis
The onset of a variety of vague symptoms such as lower abdominal pain, constipation or dyspepsia or anorexia with weight loss, the onset of menstrual disorders or urinary symptoms, abdominal bloating, pain during intercourse, fatigue and depression can be a hint and may prompt further investigations.
 
Sometimes patients will present with a clear abdominal mass, ascites or even pleural effusions.

Differential diagnoses include benign and functional cysts, endometriosis and other cancers. Sometimes a primary gastrointestinal cancer is found to be the origin of an ovarian process (Krukenberg tumour).

At present there is no established way of effectively screening women for ovarian cancer. There are hopes that the UK Collaborative Trial of Ovarian Cancer Screening, which is running until 2010 will result in more detailed knowledge with regards to screening and early detection of ovarian cancer.

The current state is that high risk women should be referred for genetic counselling, especially for the cancer genes BRCA1 and 2. However, it is unclear if this prevents the statistical mortality.

Regular ultrasound (preferably transvaginal) and pelvic examinations, as well as CA-125 tumour marker checks seem to be of mixed effectiveness in prevention.

It is well known that CA-125 can be normal in up to half of the cases of early ovarian cancer and can also be elevated in a number of other medical conditions.

Having excluded pregnancy consider requesting diagnostics including an urgent ultrasound and CXR as well as CA-125, hCG and alpha-fetoprotein as well as a general blood screen.

Many women who are found to have ovarian cysts will be anxious that they may turn malignant. Usually they can be reassured and an ultrasound scan should show that the cysts are stable or receding. If there is doubt (large number of cysts or cyst over 5cm) a referral would be indicated.

The further management and outcome of any case will depend on the histology and the staging of the tumour.

The latter is done with the FIGO classification by the International Federation of Gynaecological Oncologists. Whereas in early stages (I and II) treatment is often curative and five-year survival rate is 80 per cent, in stage III it is only about 20 per cent and stage IV around 5 per cent. Treatment options include surgery, radio- and chemotherapy.

In this case, the patient died within three months of palliative care. Her two daughters aged 22 and 20 are eligible for surveillance. 

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