Endometrial Stromal Sarcoma: Information on Causes, Diagnosis & Treatments

Cancer Uterus Younger Than Menapause Endometrial stromal sarcoma (ESS) is a rare form of uterine cancer that typically affects pre-menopausal women. It accounts for approximately one out of 100 cases involving reproductive organ cancer. The condition is a malignant form of endometrial trauma tumor that involves the connective tissue (trauma) of the endometrium instead of involving the glands.

For years, doctors have graded endometrial stromal sarcoma in two distinct categories involving low-grade and high-grade conditions. Only recently has medical science recategorized high-grade into undifferentiated and low-grade sarcoma because the condition often lacks specific differentiation and does not share the histological similarity to endometrial trauma. These two grades now include:

  • Low-Grade Endometrial Stromal Sarcoma (LGESS) that is a slow-growing form of the condition.

  • Undifferentiated Endometrial Stromal Sarcoma (High-Grade ESS) that is a more rapidly growing form of the condition.

Diagnosing the condition in its early stage provides the best chance of survival. According to the American Cancer Society, the five-year relative survival rate for endometrial stromal sarcoma, include localized – 99%, regionalized – 94%, and distant – 69%.

This means if the stromal sarcoma cells are found in their initial development stage, the patient has a 99% chance of still being alive and five years compared to finding the sarcoma after it has metastasized to distant sites in the body including the lungs and brain, where the woman has a 69% chance of being alive five years after the diagnosis.

Who Is at Risk for Endometrial Stromal Sarcoma?

Medical researchers believe that a proximally 1 million to 2 million women worldwide are affected by endometrial trauma sarcoma every year with the common age between 42 and 58 years. Where’s trauma sarcoma begins or why is poorly understood. Some scientific research indicates that there may be a chromosomal abnormality in the development and progression of stromal tumors.

Endometrial trauma sarcoma often develops in women when in their 40s and 50scompared to the age of women suffering most forms of uterine cancer. However, doctors have yet to determine all the potential risk factors involved in the condition. However, some known endometrial trauma sarcoma risk factors involve:

  • Exposure to radiation to the pelvic area or uterus that occurred five years to 25 years before.

  • Previous use of drugs like Tamoxifen and Nolvadex that affects the body’s hormone estrogen.

  • Ethnicity, especially African-American women who are 200% more likely to acquire some form of rare uterine cancer than other races.

Common Symptoms

During the initial development of endometrial trauma sarcoma, many women will experience condoms symptoms that include:

  • Swelling of the pelvis

  • Pelvic pain that might be caused by information, or a tumor (mass)

  • Vaginal discharge that might be the sign of trauma sarcoma, infection, or other benign conditions

  • Abnormally heavily and prolonged bleeding, even after menopause

In most cases, abnormal bleeding is not the result of uterine sarcoma or leiomyosarcoma but is typically associated with undifferentiated sarcoma and endometrial stromal sarcoma.

Because low-grade endometrial stromal sarcoma can develop slowly, the condition can often spread (metastasized) to distant sites outside the uterus before the doctor detects, validates or verifies a diagnosis. Because of that, it is recommended that women experiencing these symptoms be highly assertive when speaking to their health care provider to make sure they undergo an endometrial sampling test, an effective screening test that can determine or rule out the presence of uterine cancer that could be the likely cause of the unusual symptoms.

Diagnosing Endometrial Stromal Sarcoma

Many cases of endometrial stromal sarcoma are identified in diagnosed after a surgical procedure has been performed for another condition, benign fibroid tumors. As a part of the diagnosis, the doctor will conduct a family and personal medical history and determine risk factors, symptoms, or another health problem. Usually, the doctor will perform a pelvic examination and general physical exam. If cancer is suspected, the gynecologist will typically refer the patient to a specialist, likely a gynecologic oncologist who specializes in female reproductive system cancers.

The most common tests associated with endometrial tissue include:

  • Biopsy – The doctor will perform a biopsy by removing a sample of tissue from the lining of the uterus to be analyzed in a laboratory setting by a pathologist viewing at the tissue under a microscope. The removal of the tissue is usually performed by a D&C (dilation and curettage).

During a routine biopsy, the doctor will attempt to determine the cause of the bleeding that might be hyperplasia (benign endometrial overgrowth), uterine sarcoma, or another disease.

  • Endometrial Biopsy – The doctor will use a flexible, thin two that is inserted through the cervix into the uterus. The device is used to remove a small amount of endometrium (the lining of the uterus). The doctor will likely if the patient ibuprofen or some other nonsteroidal anti-inflammatory drug (NSAID) before beginning the procedure that is usually conducted in the doctor’s office.

  • Hysteroscopy – The doctor can view the interior of the uterus using a small telescope inserted through the cervix. Viewing the interior of the uterus can help identify abnormal growths that can be removed for analysis. This could include a polyp or a cancerous growth. The procedure requires a numbing medicine (local anesthetic) or in some cases, general anesthesia is used if a mass or polyp must be removed.

  • Dilation and Curettage – Sometimes, the biopsy provides inconclusive results when the doctor is unsure cancer or other condition is present. During those times, the doctor will perform a D&C (dilation and curettage) procedure using regional, general anesthesia or some form of medicated sedation to dilate the cervix and use a surgical instrument prescribing endometrial tissue to be analyzed under a microscope.

  • Endometrial Tissue Testing – By looking under the microscope, a pathologist can determine if cancer is present and identify the disease as sarcoma or carcinoma along with its grade. Determining the type of cancer provides much-needed information to the doctor who must determine the best method of treating the condition.

  • Transvaginal Ultrasound – The doctor can use a device to create sound ways for creating images of the pelvic area including the uterus to help identify the presence of tumors or cancerous growths.

  • Imaging – The doctor may order a CT (computerized tomography) scan, MRI (magnetic resonance imaging) scan, PET (positron emission tomography) scan, or chest x-ray that provides visual images of the pelvic region.

Treating the Condition

Once a diagnosis of endometrial stromal sarcoma has been verified, the doctor has numerous options for developing an effective plan of care. These include:

  • Surgical procedures

  • Radiation therapy

  • Chemotherapy therapy

  • Hormone therapy

Usually, the most successful outcome is obtained through a combination of the treatments listed above. The doctor may perform a surgical procedure to remove the cancer followed by chemotherapy and radiation to route lower the potential risk of recurring cancer. If the cancer has developed to an advanced stage, the doctor might recommend clinical trial that provides state-of-the-art treatments for the cancer based on the latest medical advancements.

However, not all patients will be the ideal candidate for clinical trial. In these cases, the doctor may recommend alternative methods for alleviating the common symptoms and discomfort associated with the condition.

Back to the Top

Sources:

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3208414/

https://mayoclinic.pure.elsevier.com/en/publications/endometrial-stromal-sarcoma-treatment-and-patterns-of-recurrence

https://www.ncbi.nlm.nih.gov/pubmed/2792908

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3385272/

https://www.cancer.org/cancer/uterine-sarcoma/about/what-is-uterine-sarcoma.html

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