Endometrial stromal sarcoma (ESS) is a rare type of cancer that develops in the connective tissue cells of the uterus, specifically in the endometrium—the inner lining of the uterus.
ESS can vary in how it presents:
- It may be confined to the uterus.
- It may start in the uterus and spread to nearby areas like the pelvis.
- It may spread beyond the uterus to more distant parts of the body.
Doctors classify ESS as either low-grade or high-grade, which indicates how aggressive the cancer is:
- Low-grade ESS: The cancer cells look somewhat like normal cells and tend to grow slowly.
- High-grade ESS: The cancer cells appear more abnormal and can grow rapidly.
This type of cancer only affects individuals with a uterus and is most commonly diagnosed in women between the ages of 40 and 60. The exact cause of ESS remains unclear. However, some studies suggest a possible link between ESS and factors such as hormone therapy, pelvic radiation, or polycystic ovary syndrome (PCOS). That said, the risk of developing ESS due to these factors is considered low, and doctors carefully weigh the benefits of treatments against any potential risks. Research is ongoing to better understand what causes ESS and how to improve treatment options.
Signs and Symptoms
The symptoms of endometrial stromal sarcoma (ESS) can vary depending on the size and location of the tumor. Some people experience multiple symptoms, while others may have none before diagnosis.
Common signs to watch for include:
- Unusual vaginal bleeding, such as:
- Bleeding between periods
- Heavier periods than usual
- Bleeding after menopause
- Unusual vaginal discharge
- Pelvic or abdominal pain
- A noticeable lump in the pelvic or abdominal area
- Changes in urination, like needing to pee more often
- Constipation
If you notice any of these symptoms, see your doctor as soon as possible.
Diagnosis
Doctors diagnose endometrial stromal sarcoma (ESS) through a series of tests, starting with a physical examination to check for any lumps. Imaging tests such as ultrasounds, X-rays, CT scans, MRI scans, or PET scans provide a clearer view of the inside of the body. Doctors often perform a transvaginal ultrasound by gently inserting a small probe into the vagina to capture detailed images of the uterus. They may also conduct a hysteroscopy, using a thin tube inserted through the vagina and cervix into the uterus to examine the area and take a biopsy if necessary. To confirm an ESS diagnosis, doctors analyze a biopsy, which involves removing and testing a sample of the tumor.
Treatment Options for ESS
Surgery
Surgery is the main treatment for endometrial stromal sarcoma (ESS), and the type of procedure depends on how far the cancer has spread. If the tumor is small and contained within the uterus, surgeons may remove just the cancer along with a small margin of healthy tissue to ensure no cancer cells are left behind.
For larger tumors, a total hysterectomy—the removal of the entire uterus—is often necessary. If the cancer has spread beyond the uterus, doctors may recommend:
- Radical hysterectomy – Removal of the uterus, nearby tissues, and the upper part of the vagina.
- Total or radical hysterectomy with bilateral salpingo-oophorectomy – Removal of the uterus, fallopian tubes, and ovaries.
If your ovaries are removed, you’ll go into surgical menopause if you haven’t already. This can bring menopause symptoms suddenly. Also, after a hysterectomy, pregnancy is no longer possible, so if you’re considering having children, talk to your doctor about fertility preservation options.
Radiotherapy
Radiotherapy uses high-energy radiation to destroy cancer cells. It may be given:
- Before surgery – To shrink the tumor and make it easier to remove.
- After surgery – To destroy any remaining cancer cells.
Chemotherapy
Chemotherapy uses powerful drugs to kill cancer cells. It’s usually recommended if there’s a high risk of the cancer coming back or if it has spread to other parts of the body.
Hormone Therapy
Since some ESS tumors grow in response to hormones, hormone therapy can help slow tumor growth by blocking or reducing hormone levels in the body.
Targeted Therapy & Immunotherapy
Newer treatments, like targeted therapy and immunotherapy, are being explored for ESS:
- Targeted therapy focuses on blocking specific molecules that help cancer cells grow and spread.
- Immunotherapy boosts the body’s immune system to help fight cancer.
Some clinical trials have shown promising results with these treatments, but more research is needed before they become widely available. If you’re interested, your doctor can tell you about any ongoing clinical trials you may qualify for.
After Treatment
After treatment, you’ll have regular follow-up appointments for several years to monitor your recovery and check for any signs of ESS returning. Your sarcoma clinical nurse specialist will provide a follow-up schedule, which typically includes discussions about any symptoms you may be experiencing, physical examinations, and imaging tests such as CT, MRI, or ultrasound scans. You may also have a chest X-ray to ensure the cancer hasn’t spread to the lungs. These appointments are an important part of your ongoing care to catch any potential issues early.
Living with ESS
ESS sometimes returns in the same place after treatment, which doctors call a local recurrence. If it comes back, starting treatment quickly is crucial, and your doctor may recommend additional surgery or radiotherapy. You can check for recurrences by watching for unusual bleeding, discharge, or other symptoms. Your doctor can explain what to look for. If you’re worried about your cancer returning, contact your doctor or nurse, who may move your follow-up appointment earlier to investigate. In some cases, ESS spreads to other parts of the body, known as metastasis or secondary cancer. Some people only receive an ESS diagnosis after the cancer has already spread, often to the lungs, which is why follow-up care includes chest X-rays. Secondary cancers can also develop in the bones, abdomen, pelvic region, or lymph nodes. Depending on your case, doctors may treat these with surgery, radiotherapy, or other therapies.
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Resource:Sarcoma.org.uk
