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Hidden dangers of undiagnosed uterine sarcoma

I was invited to present at the Austrian and Bavarian Obstetrics & Gynaecology Society in Vienna about uterine sarcomas. Uterine sarcomas are uncommon but very aggressive cancers of the womb and unintended mismanagement of these tumours can have a catastrophic effect on the patients’ chance of survival.

What are uterine sarcomas?                                                         

Uterine sarcoma is a term to describe cancers that start from tissues such as muscle, fat, bone, and fibrous tissue. Uterine sarcomas are aggressive tumours arising from the myometrium (muscle tissue) or other tissues that support the uterus. Uterine sarcoma is different from endometrial cancer (that arises from the inner lining of the womb; endometrium), but both are classified as uterine cancers (cancers of the uterus).

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Incidence

At the Queensland Centre for Gynaecological Cancer we see approximately 30 patients with uterine sarcomas every year with the incidence increasing. Uterine sarcomas are rare and account for only 1 in 20 malignancies arising from the uterus.

Risk factors and symptoms

It is not known what causes uterine sarcomas, however certain risk factors have been identified. Increasing age, long-term use of tamoxifen (e.g. for breast cancer), previous pelvic radiation treatment (e.g. for rectal cancer) and genetic mutations (hereditary kidney cancer) are associated with an increased risk for uterine sarcomas. They usually occur after menopause.

Symptoms of uterine sarcomas are unspecific. However, the majority of patients present with abnormal uterine bleeding, pain and abdominal swelling. Unfortunately, these symptoms are also shared with common and benign gynaecological conditions such as fibroids.

Against the old opinions, a rapidly growing uterus or a very large womb don’t point to the possibility of a uterine sarcoma.

Diagnosis

Conventional and modern PAP smears may pick up uterine cancer cells but in the majority of patients with these tumours it will not indicate a problem.

All patients with abnormal uterine bleeding need to have some tissue taken from the uterus to be examined under a microscope. This can be done by endometrial sampling in the surgery or by dilation and curettage (D&C) as a surgical day procedure. Unfortunately, removing tissue from the uterine cavity will still fail to diagnose rare cancers of the womb in up to 50% of patients.

Diagnosis by medical Imaging (for e.g. CT, MRI), blood tests, and pelvic exams are unreliable. Unfortunately, a significant percentage of uterine sarcomas are diagnosed incidentally following a hysterectomy or a myomectomy due to lack of any reliable screening options.

Due to the unspecific symptoms and because medical imaging is unreliable, unfortunately, only half of all uterine sarcomas will be diagnosed prior to surgery.

Morcellation

During laparoscopic surgery it will always be impossible to remove a normally-sized uterus intact through the small, 5 mm to 10 mm keyhole incisions. Morcellation means that a large mass (such as the uterus) is reduced in size so that smaller pieces of the specimen can be removed from the pelvis or the abdomen.

Morcellation can be performed during laparoscopic, vaginal or open/abdominal surgery through an incision.

In Europe I had to learn that an unacceptably high number of gynaecologists still find it acceptable to morcellate large pelvic masses, including a uterus, without any protection devices.

However, if a uterine sarcoma remained undetected, and it got morcellated without any protection, it will ‘spill’ into the perioneal surface of the pelvis and abdomen during morcellation, thus spreading cancerous cells throughout the body. This would have dreadful consequences for the patient, as anyone could imagine.

As there is no reliable method to screen for uterine sarcomas prior to surgery, it is strongly recommended gynaecologists abstain from any uncontained morcellation. The use of a power morcellator should be limited to those cases where the morcellator is operated within a containment bag to protect specimens from spillage and possibly spreading undetected cancer (potentially undiagnosed uterine sarcomas). Gynaecological surgeons need to be familiar with various surgical techniques to maintain the intactness of specimens upon their extraction from the pelvis.

Adjunct Treatment

Concerningly, uterine sarcomas do not respond well to any type of adjuvant treatment (chemotherapy or radiation). Patients will solely rely on surgery for treatment.

Is a laparoscopic hysterectomy out of question for patients with a large uterus?

No, even patients with a large uterus can have a laparoscopic hysterectomy. However, the surgeon needs to pay particular attention to remove the uterus intact (in one piece) or morcellate it within the confinements of a strong bag, which avoids spillage of potential tumour into the abdomen and pelvis.

Patients and gynaecologists need to be concerned about the possibility of an undiagnosed (and undiagnosable) tumour especially in postmenopausal woman. Unaided morcellation, regardless of whether it is performed through vaginal, open or laparoscopic surgery needs to be a thing of the past.

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