What are the other Names for this Condition? (Also known as/Synonyms)

• Endometrial Stromal Tumour
• EST (Endometrial Stromal Tumor)
• Uterine Endometrial Stromal Tumor

What is Endometrial Stromal Tumor? (Definition/Background Information)

• Endometrial Stromal Tumors (ESTs) constitute a rare group of benign and malignant tumors of the uterus. The tumors are seen in women of a wide age category, but most are observed around or following menopause
• Endometrial Stromal Tumors constitute the following set of tumors:
  • Endometrial stromal nodule (ESN): It is a rare and benign tumor
  • Low-grade endometrial stromal sarcoma (LG-ESS): It is a tumor of low-grade malignancy
  • High-grade endometrial stromal sarcoma (HG-ESS): It is a tumor of high-grade malignancy
  • Undifferentiated uterine sarcoma (UUS): It is the most aggressive form of endometrial stromal sarcomas
  • Uterine tumors resembling ovarian sex cord tumor (UTROSCT): These are ‘mostly’ benign tumors
• The tumors are known to be caused by genetic mutations and chromosomal aberrations. Risk factors for Endometrial Stromal Tumors include estrogen therapy, tamoxifen therapy, and polycystic ovary syndrome
• The signs and symptoms of Endometrial Stromal Tumor include unusual vaginal bleeding, abdominal pain, and sensation of pressure in the pelvic area. The complications are dependent upon whether the tumor is benign or malignant. Advanced stage malignancies are observed to metastasize to local and distant sites
• In most cases, treatment for Endometrial Stromal Tumor is through surgery. Additional treatment measures, that include radiation therapy and chemotherapy, may be provided for malignant tumors
• The prognosis of Endometrial Stromal Tumor mostly depends on the type of tumor, the nature of the tumor, and the stage of the tumor (if it is malignant). Generally, benign tumors have an excellent prognosis; while, the prognosis of malignant tumors may be guarded

Who gets Endometrial Stromal Tumor? (Age and Sex Distribution)

• Endometrial Stromal Tumors may be present in adult women of any age; both pre- and post- menopausal women may be affected
• There is no geographical, racial, or ethnic preference observed

What are the Risk Factors for Endometrial Stromal Tumor? (Predisposing Factors)

The following may be potential risk factors for Endometrial Stromal Tumors:

• Tamoxifen therapy
• Unopposed estrogen therapy
• Radiation to the pelvic region
• Polycystic ovarian syndrome (PCOS)

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one’s chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Endometrial Stromal Tumor? (Etiology)

The cause of development of Endometrial Stromal Tumor is related to genetic abnormalities. The tumor is known to originate from the endometrial stroma, in many cases. The gene mutations and chromosomal alterations are specific to the subtype of EST.

• JAZF1-SUZ12 fusion gene due to chromosomal translocation abnormality, namely t(7;17)(p21;q15), is observed in ESN and LG-ESS
• YWHAE-FAM22 fusion gene due to chromosomal translocation, namely t(10;17)(q22;p13), is observed in HG-ESS
• Additions and deletions on certain chromosomes have been identified in UUS

Unlike other Endometrial Stromal Tumors, UTROSCT tumors do not exhibit the characteristic JAZF1-SUZ12 fusion gene. This may indicate that these tumors are not of endometrial stromal origin.

What are the Signs and Symptoms of Endometrial Stromal Tumor?

Endometrial Stromal Tumors may be benign or malignant. Some women may not show any signs and symptoms. In others, the signs and symptoms of EST may include:

• The presence of a solitary, small or large tumor; the majority are between 5-10 cm in size
• Generally, malignant tumors have poorly-defined boundaries; while, benign tumors are mostly well-defined
• Sensation of pelvic mass or enlarged uterus
• Most of the tumors are located intramurally (i.e., found within the uterine walls)
• In some cases, polypoid tumors are observed
• Heavy or abnormal bleeding from the uterus and abdominal pain are both common symptoms in women with EST
• Signs and symptoms may also occur from large-sized tumors causing compression of adjoining uterine structures including frequent urination and lower back pain
• Additionally, in malignant tumors, the following may be observed:
  • Local infiltration, into the surrounding tissues
  • Tumor necrosis and hemorrhage is observed
  • The tumors can invade into the myometrium and vascular system of lymph nodes (lymphovascular spaces)
  • Weight loss, weakness, and lethargy
• Pain during sexual intercourse

How is Endometrial Stromal Tumor Diagnosed?

The diagnosis of Endometrial Stromal Tumor may involve:

• Complete physical examination with thorough evaluation of medical history
• Ultrasound scan of the pelvis: It is a noninvasive procedure that uses high frequency sound waves to produce real-time images
• Abdominal and pelvic CT scan: It is a noninvasive procedure that provides more details of soft tissues, blood vessels, and internal organs
• Pelvic MRI scan: It is a noninvasive medical test that uses a powerful magnetic field to produce images of soft tissues, bones, organs, and all other internal structures of the abdomen and pelvis
• Blood tests that include:
  • Complete blood count (CBC) to detect the cause of anemia
  • Determination of estrogen/progesterone levels in blood
• Hysteroscopy: This procedure involves placing a probe through the cervix to examine the cavity of the uterus. This exam is helpful in detecting submucosal tumors
• Hysterosalpingography: It is usually performed in individuals with infertility. In this procedure, the structure of the uterus and fallopian tubes are studied by using a dye and X-ray images
• Needle biopsy of tumor: A needle biopsy may not be helpful, because one may not be able to visualize the different morphological areas of the tumor. Hence, a needle biopsy as a diagnostic tool has certain limitations, and an open surgical biopsy is preferred
• Open biopsy of tumor: A tissue biopsy is performed and sent to a laboratory for a pathological examination, who examines the biopsy under a microscope. After putting together clinical findings, special studies on tissues (if needed) and with microscope findings, the pathologist arrives at a definitive diagnosis

Note:

• A differential diagnosis to eliminate other tumor types is considered, before arriving at a definitive diagnosis of EST
• A dilation and curettage (D&C) of the uterus or an endometrial biopsy is not helpful in establishing a proper classification of ESTs such as low-grade and high-grade endometrial stromal sarcomas
• Extensive sampling during a pathological examination can help diagnose Endometrial Stromal Tumors and exclude other benign and malignant tumors

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Endometrial Stromal Tumor?

The possible complications of Endometrial Stromal Tumor include:

• Stress and anxiety due to cancer of the uterus
• Infertility
• Heavy and prolonged menstrual bleeding may result in anemia
• Polypoid tumors may undergo mechanical injury, such as torsion or twisting, which can result in excruciating pain
• Such polyp-shaped tumors are known to cause cervical prolapse, in some cases
• Some tumors may grow to large sizes, and even grow out of the uterus affecting adjoining reproductive organs
• Malignant tumors are known to metastasize to various local and regional sites, resulting in site-specific signs and symptoms
• Recurrence of the tumor due to its partial or incomplete surgical removal
• Side effects of chemotherapy (such as toxicity) and radiation
• Sexual dysfunction can take place as a side effect of surgery, chemotherapy, or radiation therapy

How is Endometrial Stromal Tumor Treated?

The treatment measures for Endometrial Stromal Tumor may include the following:

In case of benign tumors:

• Asymptomatic or small-sized benign tumors may not require any treatment; in such cases, the healthcare provider may choose to periodically observe and monitor the tumor
• A simple surgical excision and removal of the entire tumor is normally sufficient treatment
• In women who have completed their family, a hysterectomy, which is the surgical removal of a part of or the entire uterus, may be undertaken

In case of malignant tumors:

• Wide surgical excision with removal of the entire tumor is the standard treatment mode
• When the tumor is at an inaccessible location, or is unsafe for surgical intervention, non-invasive procedures, such as chemotherapy and radiation therapy may be considered
• Hormone therapy is employed in women with advanced endometrial stromal sarcomas that have metastasized beyond the uterus
• Many patients with metastatic or locally advanced tumors may be referred for clinical trials for experimental treatment options
• Post-operative care is important: A minimum activity level is to be ensured until the surgical wound heals

Once treatment is complete, it is recommended that the patient schedule regular check-ups (typically for a long duration of time), based on the recommendation of the specialist treating them.

How can Endometrial Stromal Tumor be Prevented?

Current medical research has not established a method of preventing Endometrial Stromal Tumor. However, the following general factors may be considered to reduce the risk for tumor development:

• Address any condition causing hormonal imbalance in the body
• Maintain weight through proper diet modification and physical exercises, if you are overweight/obese
• Avoid alcohol consumption or limit its intake
• Have a balanced diet that is not high in meat and low in vegetables; a balanced diet can also help avoid any mineral or vitamin deficiencies in the body
• Regular medical screening at periodic intervals with blood tests, radiological scans, and physical examinations are mandatory, due to risk of recurrence and metastasizing potential of the tumor. Often several years of active vigilance may be necessary

What is the Prognosis of Endometrial Stromal Tumor? (Outcomes/Resolutions)

• In many cases, the prognosis of benign Endometrial Stromal Tumors is generally excellent on surgical excision and removal of the tumor
• The prognosis of malignant Endometrial Stromal Tumor mostly depends on the cancer stage, and is generally good for tumors diagnosed in the initial stages. However, a sizable majority are diagnosed during stage III or IV (advanced stages), in which case, the prognosis is guarded
• In general, the prognosis of malignant EST depends upon a set of several factors that include:
  • The size of the tumor and the extent of its invasion: Individuals with small-sized tumors fare better than those with large-sized tumors
  • Stage of cancer: With lower-stage tumors, when the tumor is confined to site of origin, the prognosis is usually excellent with appropriate therapy. In higher-stage tumors, such as tumors with metastasis, the prognosis is poor
  • Cell growth rate of the cancer
  • Overall health of the individual: Individuals with overall excellent health have better prognosis compared with those with poor health
  • Age of the individual: Older individuals generally have poorer prognosis than younger individuals
  • Individuals with bulky disease have a poorer prognosis
  • Involvement of the regional lymph nodes, which can adversely affect the prognosis
  • Involvement of vital organs may complicate the condition
  • The surgical respectability of the tumor (meaning, if the tumor can be removed completely)
  • Whether the tumor is occurring for the first time, or is a recurrent tumor. Recurring tumors have worse prognosis compared to tumors that do not recur
  • Response to treatment: Tumors that respond to treatment have better prognosis compared to tumors that do not respond to treatment
  • Progression of the condition makes the outcome worse
• An early diagnosis and prompt treatment of the tumor generally yields better outcomes than a late diagnosis and delayed treatment
• The combination chemotherapy drugs used, may have some severe side effects (like cardio-toxicity). This chiefly impacts the elderly adults, or those who are already affected by other medical conditions. Individuals, who tolerate chemotherapy sessions better, generally have better outcomes

Additional and Relevant Useful Information for Endometrial Stromal Tumor:

• Removal of the uterus will cause the regular menstrual bleeding to stop. This also means that a woman may not have children after uterus removal; though, sexual intercourse is still possible 
• Hysterectomy is a surgical procedure characterized by the surgical removal of the uterus

The following link will help you understand hysterectomy surgical procedure:

http://www.dovemed.com/common-procedures/procedures-surgical/hysterectomy-abdominal-with-salpingo-oophorectomy/