Endometrial Stromal Sarcoma - Clinical Implications

What Is Endometrial Stromal Sarcoma?

ESS is a rare tumor affecting the endometrium (inner lining) of the uterus and is malignant (cancerous). These are uncommon uterine cancers that arise from the stromal cells of the inner lining of the uterus. These cells are normally supportive and connective tissue cells that maintain the structural integrity of the endometrium. This article highlights the different variants of ESS, causes, symptoms, and management of ESS.

What Is the Incidence of Endometrial Stromal Sarcoma?

Endometrial stromal sarcoma is a very rare form of uterine cancer. These cancers affect mostly adults in the age range of 40 to 55 years of age. The incidence of low-grade ESS is higher among all the variants. The incidence of ESS is around one to two cases per one lakh females.

What Is the Prevalence of Endometrial Stromal Sarcoma?

The prevalence rate of endometrial stromal sarcoma is quite low. It accounts for almost 0.2 percent of overall uterine cancers.

What Are the Subtypes of Endometrial Stromal Sarcoma?

ESS has two different variants:

• Low-grade ESS: Low-grade ESS usually has cancer cells that are not much different from the normal stromal cells and are difficult to differentiate. These grade tumors grow and spread quite slowly.

• High-grade ESS: In the case of high-grade ESS, the cancerous cells present completely different characteristics than those of normal cells and can be easily differentiated. This type of cancer grows and spreads at a faster rate.

What Are the Symptoms of Endometrial Stromal Sarcoma?

The symptoms associated with endometrial stromal sarcoma are:

• Bleeding from the uterus (such as bleeding between menstrual periods).

• Pain in the pelvic region.

• Enlargement of the uterus.

• Abnormal vaginal discharge.

• Bleeding after menopause (after cessation of menstrual cycle).

• Abdominal pain.

• A lump can be palpated in the region of the pelvis or stomach.

• Increased frequency of urination.

• Constipation (stomach upset).

If any of these symptoms are observed, visiting the healthcare professional as soon as possible is advised.

How Is Endometrial Stromal Sarcoma Diagnosed?

Endometrial stromal sarcoma can be diagnosed by:

• History and Clinical Examination: A thorough clinical history of the patients for the presenting signs and symptoms should be recorded. The healthcare specialist usually examines clinically for the presence of the lump in the abdomen or pelvic region.

• Radiographic Analysis: The use of multiple radiographic modalities such as PET (positron emission tomography) scan, computed tomography (CT) scan, and magnetic resonance imaging (MRI) can be used to check the severity and spread of the tumor.

• Hysteroscopy: Hysteroscopy is performed by the specialist doctors by inserting the thin tube through the vagina, cervix, and into the uterus. This technique helps to detect any signs of uterine cancer and the location of the tumor. A biopsy (collecting a sample of diseased tissue for pathological examination) can be taken if needed during this process.

• Tissue Biopsy: In this technique, a tissue sample is usually derived from the tumor surgically and sent to the lab for pathological examination. This helps in reaching the exact diagnosis of ESS.

How Is Endometrial Stromal Sarcoma Managed?

The following methods can treat the endometrial stromal sarcoma:

• Surgical Therapy: Surgical therapy is the main approach for the management of ESS. The decision on the type and technique of surgery is made based on tumor size and the extent of the spread. Different surgical modalities used are:

1. Surgical Resection: This method is usually followed for small-size uterine tumors. A surgeon usually removes the tumor from the uterine lining surgically using the surgical blade and leaves the healthy margin. Along with the cancerous tissue line, some healthy tissue is also removed to ensure the cancerous tissue is removed completely and to avoid the recurrence of the cancer.

2. Total Hysterectomy: This method is usually employed for larger uterine cancers. In this procedure, the surgeon usually removes the entire uterus to avoid cancer spreading to other areas of the body.

3. Radical Hysterectomy: This technique is usually followed by the surgeon when the cancer is very large and has also spread to nearby tissues. In this procedure, the uterus, surrounding tissue, and the upper part of the vagina are removed surgically by the surgeon.

4. Radical Hysterectomy with Bilateral Salpingo-Oophorectomy: This procedure is usually opted for by the surgeon when the cancerous tissue is spread to different surrounding tissues and organs. In this procedure, the surgeon removes the uterus, fallopian tubes, and ovaries surgically. After the ovaries are removed during this technique, the individual will go through menopause, also called “surgical menopause. There are no chances of females getting pregnant after hysterectomy surgical procedures. It is advised to talk to healthcare providers about possible fertility treatments.

• Radiotherapy: In this method, high-energy beams are utilized to destroy the cancerous cells. Radiotherapy is usually performed in two sessions, one before the surgery to shrink the tumor size and one after the surgery to kill any remnants of tissues left after the surgery.

• Chemotherapy: Anticancer agents are used in this procedure to destroy the cancerous cells. This method is mostly used in cancers that are high-grade or metastasized (spread to distant parts).

• Hormonal Therapy: Hormones are responsible for the growth of ESS tumors. By utilizing hormonal therapy, the specific hormone responsible for the growth of tumors is blocked. Hormonal therapy helps to slow down the growth of ESS tumors.

• Targeted Therapies: Targeted therapies are one of the recent approaches used to restrict cancer cells from growing and spreading.

• Immunotherapy: This therapy helps the body's immune system to recognize and kill the cancer cells.

What Is the Prognosis of Endometrial Stromal Sarcoma?

The prognosis of ESS completely relies on:

• The grading of the tumor.

• Severity of spread of the tumor.

• The stage at which the tumor is diagnosed.

• Clinical outcome of the treatment.

Ideally, low-grade tumors have a better prognosis as compared to higher-grade tumors.

Conclusion

ESS is a rare type of cancer originating from the stromal cells of the endometrium. Each type of ESS has distinct characteristics and prognoses. Regular follow-up and collaboration with a healthcare team are crucial for monitoring and managing the condition. As research and understanding of ESS continue to evolve, ongoing advancements in treatment options may offer improved outcomes for individuals affected by this rare uterine cancer.