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

• Cervical Leiomyosarcoma
• Leiomyosarcoma of Cervix
• LMS of Cervix

What is Leiomyosarcoma of Uterine Cervix? (Definition/Background Information)

• Leiomyosarcoma (LMS) is a rare type of connective tissue cancer, accounting for 5-10% of all soft tissue sarcomas (a type of cancer). Even though rare, Leiomyosarcoma of Uterine Cervix is a common subtype of cervical sarcoma found in middle-aged women
• Experimental analysis point to the cell line origin for leiomyosarcoma being smooth muscle cells. Smooth muscles are muscles that are not voluntarily controlled. Due to the bounty of smooth muscle throughout the body, any individual is susceptible to LMS, although the elderly are more prone to the condition
• There are currently no established and specific risk factors, causes, or preventive methods for Leiomyosarcoma of Uterine Cervix
• The signs and symptoms of Cervical LMS include unusual vaginal discharges, bleeding, and sensation of pressure in the pelvic area. The complications are dependent upon the stage of the cancer and may also include treatment complications
• Treatment for Leiomyosarcoma of Uterine Cervix is mainly through surgery and other supplementary treatment measures. The prognosis of Cervical Leiomyosarcoma depends on the cancer stage and overall health of the individual

Who gets Leiomyosarcoma of Uterine Cervix? (Age and Sex Distribution)

• Typically, women between the ages of 30 and 60 years are susceptible to Leiomyosarcoma of Uterine Cervix
• There are no known geographical localizations; this cancer type is found worldwide
• Leiomyosarcoma of Cervix is the most common primary sarcoma of the cervix, even though generally cervical sarcomas are rare

What are the Risk Factors for Leiomyosarcoma of Uterine Cervix?

While there are no well-established risk factors for Leiomyosarcoma of Uterine Cervix, there are a few leading theories:

• Certain inherited genetic traits are believed to increase the risk
• High-dose radiation exposure to the pelvis, such as pelvic radiation therapy, is believed to increase the risk for leiomyosarcoma
• Being born with an abnormal copy of the RB (retinoblastoma) gene may increase one’s risk. Retinoblastoma, a type of eye cancer, may also arise from an abnormal copy of this gene
• Immunocompromised patients infected by Epstein-Barr virus seem to be predisposed to LMS. The reason for this is not understood, yet there seems to be a definite correlation between the viral infection and the arising of multiple, synchronized leiomyosarcomas
• Some uterine sarcomas have been associated with the use of the drug tamoxifen. This drug is given for the treatment of breast cancer

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases ones 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 Leiomyosarcoma of Uterine Cervix? (Etiology)

Currently, there are no known causes for Leiomyosarcoma of Uterine Cervix.

• As smooth muscles are found widely throughout the body, any individual is susceptible to leiomyosarcoma
• However, due to the rarity of the cancer, it is difficult to determine what exactly leads to the formation of Cervical LMS

What are the Signs and Symptoms of Leiomyosarcoma of Uterine Cervix?

The signs and symptoms of Leiomyosarcoma of Cervix may include:

• Unusual feeling of fullness in the pelvic region
• Pain in the pelvic or abdominal region
• Vaginal bleeding, vaginal discharge
• The tumors may grow to large sizes and be bulky expanding masses
• Large tumors may project into the uterus or vagina
• The average size of the tumor may be around 10-12 cm; the tumors are poorly-circumscribed
• Frequent urination or urinary retention may be noted

Less frequent signs and symptoms of Cervical LMS include:

• Weight loss
• Weakness, lethargy
• Fever

How is Leiomyosarcoma of Uterine Cervix Diagnosed?

A diagnosis of Leiomyosarcoma of Uterine Cervix may be made by using the following resources:

• Preliminary examination composed of:
  • Complete physical examination including pelvic exam
  • Evaluation of medical (and family) history
• Initial diagnosis that is made by:
  • Transvaginal ultrasound of the uterus can provide an image of the cervix and surrounding pelvic organs
  • MRI scans can be used to observe if a cervical tumor has the characteristics of cancer, along with visualizing the cancer spread (if it has spread to other areas)
  • Plain radiographs of the chest can provide evidence if the tumor has spread to the lungs
  • CT scans are rarely used in diagnosing cervical cancer, but can be used to determine if metastasis has occurred
• A hysteroscopy may be performed to visualize and simultaneously perform the biopsy of any abnormal growth within the uterus. A hysteroscopy is performed with the aid of a tiny telescope through the uterus that allows a visualization of the area
• A cervical biopsy may be necessary to determine, if the tumor present is a leiomyosarcoma, or a different soft tissue sarcoma. In the tissue biopsy procedure, the physician removes a sample of the tissue and sends it to the laboratory for a histopathological examination. The pathologist examines the biopsy under a microscope and arrives at a definitive diagnosis after a thorough evaluation of the clinical and microscopic findings, as well as by correlating the results of special studies on the tissues (if required)

Cone biopsy or conization:  This procedure is only helpful if the tumors are small enough to be completely excised by conization surgical procedure. In a majority of cases of Cervical Leiomyosarcoma, the tumor is diagnosed in the advanced stages, and hence, conization is rarely helpful.

• A cone-shaped piece of tissue is removed from the cervix during conization
• The exocervix (the outer part) forms the base of this cone, while the endocervix (the inner part) forms the apex

Two methods can be used to obtain a cone biopsy specimen:

• Loop electrosurgical procedure (LEEP, LLETZ): After numbing the area with a local anesthetic, a wire loop heated with electricity is used to remove a tissue specimen. This procedure, lasting about 10 minutes, may cause some cramping and mild-to-moderate bleeding, for a few weeks
• Cold knife cone biopsy: This procedure is performed, either under general anesthesia or under spinal anesthesia. The tissue sample is removed using a surgical scalpel or through laser

Note: Pap smear is not a good screening tool for Cervical Leiomyosarcoma.

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 possible Complications of Leiomyosarcoma of Uterine Cervix?

The possible complications of Leiomyosarcoma of the Uterine Cervix include:

• The rarity of the condition may cause a delayed diagnosis leading to metastasis
• Metastasis is likely to occur in the early stages of Leiomyosarcoma of Uterine Cervix
• The tumor may also adversely impact adjoining/surrounding structures, such as the nerves and joints, leading to discomfort or a loss of feeling
• Side effects of chemotherapy (such as toxicity) and radiation
• Sexual dysfunction can take place as a side effect of surgery, chemotherapy, or radiation therapy
• Recurrence of the cancer following incomplete surgical removal

How is Leiomyosarcoma of Uterine Cervix Treated?

Once a diagnosis of cervical cancer has been made, the extent to which the tumor has spread is assessed. This is called staging.

Following is the staging protocol for cervical cancer, according to the American Joint Committee on Cancer (AJCC), updated July 2016:

Stage 0 cervical cancer (carcinoma in situ):

• In this stage, abnormal cells are found in the innermost lining of the cervix
• These abnormal cells may become cancer and spread into nearby normal tissue

Stage I cervical cancer: The cancer is found only in the cervix. Stage I is divided into stages IA and IB, based on the amount of cancer that is found.

• Stage IA: A very small amount of cancer that can only be seen with a microscope is found in the tissues of the cervix
  • In stage IA1, the cancer is not more than 3 mm deep and not more than 7 mm wide
  • In stage IA2, the cancer is more than 3 mm, but not more than 5 mm deep; it is not more than 7 mm wide
• Stage IB: It is divided into stages IB1 and IB2, based on the size of the tumor
  • In stage IB1, the cancer can only be seen with a microscope and is more than 5 mm deep and more than 7 mm wide; or the cancer can be seen without a microscope and is not more than 4 cm
  • In stage IB2, the cancer can be seen without a microscope and is more than 4 cm

Stage II cervical cancer: The cancer has spread beyond the uterus, but not onto the pelvic wall (the tissues that line the part of the body between the hips), or to the lower third of the vagina. Stage II is divided into stages IIA and IIB, based on how far the cancer has spread.

• Stage IIA: The cancer has spread beyond the cervix to the upper two-thirds of the vagina, but not to tissues around the uterus
• Stage IIA is divided into stages IIA1 and IIA2, based on the size of the tumor
  • In stage IIA1, the tumor can be seen without a microscope and is not more than 4 cm in size
  • In stage IIA2, the tumor can be seen without a microscope and is more than 4 cm in size
• Stage IIB: The cancer has spread beyond the cervix to the tissues around the uterus, but not onto the pelvic wall

Stage III cervical cancer: The cancer has spread to the lower third of the vagina, and/or onto the pelvic wall, and/or has caused kidney problems. Stage III is divided into stages IIIA and IIIB, based on how far the cancer has spread.

• Stage IIIA: The cancer has spread to the lower third of the vagina, but not onto the pelvic wall
• Stage IIIB: The cancer has spread to the pelvic wall; and/or the tumor has become large enough to block the ureters (the tubes that connect the kidneys to the urinary bladder). This blockage can cause the kidney to enlarge or stop working

Stage IV cervical cancer: In stage IV, the cancer has spread beyond the pelvis, or can be seen in the lining of the bladder and/or rectum, or has spread to other parts of the body. Stage IV is divided into stages IVA and IVB, based on where the cancer has spread.

• Stage IVA: The cancer has spread to the nearby organs, such as the urinary bladder or rectum
• Stage IVB: The cancer has spread to other parts of the body, such as to the lymph nodes, lung, liver, intestine, or bone

(Source: Stages of Cervical Cancer, July 2016, provided by the National Cancer Institute at the National Institutes of Health; U.S. Department of Health and Human Services)

The treatment of Leiomyosarcoma of Uterine Cervix involves surgery, which is the most common treatment option considered.

• Surgical measures include:
  • Conization procedure, besides helping with the biopsy, can also help in treating very early-stage cervical cancers in women, who want to preserve their childbearing ability
  • Radical trachelectomy: The surgeon removes the cervix, upper part of the vagina, and nearby lymph nodes, while preserving the ability to have children
  • Hysterectomy: In this procedure, the uterus and cervix are removed. This is done by making an incision on the abdomen (termed abdominal hysterectomy), or through the vagina (termed vaginal hysterectomy), or by using a laparoscope (termed laparoscopic hysterectomy). Surgery is performed under general or epidural anesthesia, though the ability to have children is lost. Complications, such as bleeding, infection, or damage to the urinary tract, or the intestinal system may occur in rare cases
  • Radical hysterectomy: The uterus, cervix, the upper part of the vagina and tissues, next to the uterus are removed. Additionally, some pelvic lymph nodes may also be surgically taken out. The surgery is performed under anesthesia and may be carried out, via an incision made on the abdomen or by using laparoscopy. With this invasive procedure, the ability to have children is lost. Rarely, complications such as bleeding, infection, or damage to the urinary tract or the intestinal system, may occur. Removal of lymph nodes may lead to swelling of legs (lymphedema)
  • Pelvic exenteration: The uterus, tissues surrounding the uterus, cervix, pelvic lymph nodes, and the upper part of the vagina, are removed. In addition, depending on the tumor spread, the remainder of the vagina, the bladder, rectum, and a part of the colon, may also be removed. Recovery from this surgery takes a long period
• Although Leiomyosarcoma of Cervix has the ability to spread to the surrounding lymph nodes, a lymph node resection has not yet proven to be therapeutic
• Other than surgery, LMS provides a treatment challenge due to the observed resistance to chemotherapy and radiation therapy
• Currently, clinical trials on adjuvant chemotherapy and combinational chemotherapy, as secondary treatment to hysterectomy, are showing promising results on reducing the risk of relapse

In addition to traditional adjuvant therapies, the following techniques are currently being investigated:

• Immunotherapy aims to stimulate the patient’s immune system to recognize and destroy the cancer cells. It includes:
  • Antigen vaccines
  • DNA vaccines
  • Viral therapy
  • Gene therapy

Once treatment is complete, it is recommended that the individual schedule regular check-ups, based on the recommendation of the specialist treating them. 

How can Leiomyosarcoma of Uterine Cervix be Prevented?

• There are currently no known methods of preventing Leiomyosarcoma of Uterine Cervix
• Due to its high metastasizing potential and recurrence rate, regular medical screening at periodic intervals with blood tests, scans, and physical examinations, are mandatory for those who have already been treated for this tumor

What is the Prognosis of Leiomyosarcoma of Uterine Cervix? (Outcomes/Resolutions)

• The prognosis for Leiomyosarcoma of Cervix 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
• While removal of the uterus is the most commonly practiced treatment, recurrence of leiomyosarcoma may occur, especially in the pelvic area
• 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
• It is important to schedule and attend follow-up appointments with the healthcare provider. Many patients with metastatic or locally advanced tumors may be referred for clinical trials for experimental treatment options

Additional and Relevant Useful Information for Leiomyosarcoma of Uterine Cervix:

• 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 
• Although leiomyosarcomas are rare cancer forms, there are many online discussion groups, local groups, and sarcoma centers available to provide help and support