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

• LMS of Vagina
• Vaginal Leiomyosarcoma
• Vaginal LMS

What is Leiomyosarcoma of Vagina? (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 Vagina is the most common subtype of adult vaginal 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 Vagina
• The signs and symptoms of Vaginal Leiomyosarcoma include urination difficulties, pain during sex, and the presence of a mass in the vagina. The complications are dependent upon the stage of the cancer and may also include treatment complications
• Treatment for Leiomyosarcoma of Vagina is mainly through surgery and other supplementary treatment measures. The prognosis of Vaginal Leiomyosarcoma depends on the cancer stage and overall health of the individual. In general, the prognosis of individuals with the tumor is guarded

Who gets Leiomyosarcoma of Vagina? (Age and Sex Distribution)

• Typically, women between the ages of 22 and 86 years are susceptible to Leiomyosarcoma of Vagina; the peak age range being in the 5th decade (40-49 years)
• There are no known geographical localizations; this cancer type is found worldwide
• Leiomyosarcoma of Vagina is the most common primary sarcoma of the vagina, even though vaginal sarcomas are rare. About 2-3% of all vaginal tumors are leiomyosarcomas
• Current medical literature indicates that only up to 200 cases of Vaginal Leiomyosarcomas have been recorded

What are the Risk Factors for Leiomyosarcoma of Vagina? (Predisposing Factors)

While there are no well-established risk factors for Leiomyosarcoma of Vagina, 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 Vagina? (Etiology)

Currently, there are no known causes for Leiomyosarcoma of Vagina.

• 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 Vaginal LMS

Vaginal Leiomyosarcoma is not a sexually-transmitted disease/condition.

What are the Signs and Symptoms of Leiomyosarcoma of Vagina?

The signs and symptoms of Leiomyosarcoma of Vagina may include:

• Presence of an enlarging vaginal tumor mass; there may be ulceration of the tumor surface
• Bleeding from the rectum
• Pain during sex
• Pain in the pelvic or abdominal region
• Vaginal bleeding, vaginal discharge
• The average size of the tumor may be around 3-5 cm; the tumors are poorly-circumscribed
• The tumors may grow to large sizes and be bulky expanding masses; large tumors may project into the cervix or outside the vulva
• Urinary tract associated signs and symptoms such as frequent urination, painful urination, blood in urine (hematuria), etc.
• If the urinary bladder is involved, then it may obstruct the bladder and lead to retention of urine

Less frequent signs and symptoms of Vaginal LMS include:

• Weight loss
• Weakness, lethargy
• Fever

How is Leiomyosarcoma of Vagina Diagnosed?

A diagnosis of Leiomyosarcoma of Vagina 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 vagina and surrounding pelvic organs
  • MRI scans can be used to observe if a vaginal 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 vaginal cancer, but can be used to determine if metastasis has occurred
• Colposcopy:
  • The cervix (including the vagina) is examined with an instrument, called a colposcope. This helps the physician get a magnified view of the cervix
  • In order for this procedure to be performed, the individual has to lie on a table, as for a pelvic exam. An instrument, called the speculum, is placed in the vagina to keep the opening apart, in order to help the physician visualize the cervix. The colposcope is then used to get a magnified view of the inside
• 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 vaginal 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)

Note: Pap smears are not helpful as screening tools for vaginal cancers, because a Pap smear examines cells from the cervix and not from the vagina. Nevertheless, regular Pap smears are still important because a healthcare provider typically performs a pelvic exam at the time of collecting a Pap smear specimen. Incidentally, during such an examination, if there is a mass in the vaginal area, it may be revealed.

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 Vagina?

The possible complications of Leiomyosarcoma of the Vagina include:

• Ulceration of large tumor masses may get secondarily infected with bacteria or fungus
• 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 Vagina, since it is an aggressive cancer
• The tumor may also adversely impact adjoining/surrounding structures, such as the nerves and joints, leading to discomfort or a loss of feeling
• Topical therapy for initial stage cancers can result in side effects such as severe vaginal and vulvar irritations
• 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 is known to be frequent; recurrence is commonly observed in the pelvic region

How is Leiomyosarcoma of Vagina Treated?

Once a diagnosis of vaginal cancer has been made, the extent to which the tumor has spread is assessed, known as staging. The staging for vaginal cancer is based upon the FIGO (International Federation of Gynecology and Obstetrics) and the AJCC (American Joint Committee on Cancer) TNM staging systems.

The TNM classification for vaginal cancer is given below:

Tumor extent (T):

• Tis: Cancer cells are only in the most superficial layer of cells of the vagina without growth into the underlying tissues. This stage is also called carcinoma in situ (CIS) or vaginal intraepithelial neoplasia 3 (VaIN 3). It is not included in the FIGO system
• T1: The cancer is only in the vagina
• T2: The cancer has grown through the vaginal wall, but not as far as the pelvic wall
• T3: The cancer is growing into the pelvic wall
• T4: The cancer is growing into the bladder or rectum or is growing out of the pelvis

Lymph node spread of cancer (N):

• N0: The cancer has not spread to lymph nodes
• N1: The cancer has spread to lymph nodes in the pelvis or groin (inguinal region)

Distant spread of cancer (M)

• M0: The cancer has not spread to distant sites
• M1: The cancer has spread to distant sites

Stage grouping: Once the T, N, and M categories have been assigned, this information is combined to assign an overall stage in a process called stage grouping. The stages identify tumors that have a similar outlook and are treated in a similar way.

Stage 0 (Tis, N0, M0):

• In this stage, cancer cells are only in the top layer of cells lining the vagina (the epithelium) and have not grown into the deeper layers of the vagina
• Cancers of this stage cannot spread to other parts of the body
• Stage 0 vaginal cancer is also called carcinoma in situ (CIS) or vaginal intraepithelial neoplasia 3 (VaIN 3)
• This stage is not included in the FIGO system

Stage I (T1, N0, M0):

• The cancer has grown through the top layer of cells but it has not grown out of the vagina and into nearby structures (T1)
• It has not spread to nearby lymph nodes (N0) or to distant sites (M0)

Stage II (T2, N0, M0):

• The cancer has spread to the connective tissues next to the vagina but has not spread to the wall of the pelvis or to other organs nearby (T2)
• The pelvis is the internal cavity that contains the internal female reproductive organs, rectum, bladder, and parts of the large intestine
• It has not spread to nearby lymph nodes (N0) or to distant sites (M0)

Stage III - either of the following:

• T3, any N, M0:
  • The cancer has spread to the wall of the pelvis (T3)
  • It may (or may not) have spread to nearby lymph nodes (any N), but it has not spread to distant sites (M0)

OR

• T1 or T2, N1, M0:
  • The cancer is in the vagina (T1) and it may have grown into the connective tissue nearby (T2)
  • It has spread to lymph nodes nearby (N1), but has not spread to distant sites (M0)

Stage IVA (T4, Any N, M0):

• The cancer has grown out of the vagina to organs nearby (such as the bladder or rectum) (T4)
• It may or may not have spread to lymph nodes (any N)
• It has not spread to distant sites (M0)

Stage IVB (Any T, Any N, M1):

• Cancer has spread to distant organs such as the lungs (M1)

(Source: “The FIGO/AJCC system for staging vaginal cancer”; information provided by the American Cancer Society, February 2016)

Leiomyosarcoma of Vagina is treated using several methods depending on the stage of the cancer. The treatment options may include the following measures:

• If the tumor can be surgically removed with minimal damage and mutilation of the genitalia, then a conservative surgery is attempted. Otherwise, a combination of radiation and chemotherapy may be administered to shrink and confine the tumor, before invasive procedures are sought
• Following a surgical excision and tumor removal, a dose of chemotherapy and/or radiation therapy may be administered to kill any remaining cancer cells
• Radiation therapy and chemotherapy may be necessary if the spread of the cancer to distant sites is noted
• Advanced stage tumors with metastasis may be treated on a case-by-case basis; often symptomatic treatment with radiation and/or chemotherapy is attempted
• Very infrequently, in order to treat the more aggressive tumors, a combination of stem cell transplant with chemotherapy (high-dose) may be necessitated

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

Surgery:

• Vaginectomy: It is the surgical removal of a part of the vagina (called partial vaginectomy), or the entire vagina (called total vaginectomy), or the vagina and its surrounding affected structures/tissues may also be removed (called radical vaginectomy)
• Radical trachelectomy: The surgeon removes the cervix, upper part of the vagina, and nearby lymph nodes, while preserving the ability to have children
• If the uterus is involved, then the following may be considered:
  • 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 entire vagina, the surrounding tissues, and the pelvic lymph nodes are removed. In addition, depending on the extent of tumor spread, parts affected around the region (such as the cervix, uterus, rectum, colon, etc.) may be removed. Recovery from this surgery usually takes a long period
• Although Leiomyosarcoma of Vagina 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.

Chemotherapy:

• Medications are used to kill the tumor cells, which may be given as oral pills or injected into veins
• A combination of chemotherapy medications may be used
• Chemotherapy may be used in addition to radiation and/or surgery, to treat cancers that have spread or recurred. When chemotherapy and radiation therapy are used together, it is called concurrent chemoradiation
• Side effects of chemotherapy may include nausea, vomiting, hair loss, loss of appetite, diarrhea, fatigue, increased risk of infection, mouth sores, and easy bruising, depending on the drugs used

Radiation therapy:

• This procedure uses high-energy beams to kill the cancer cells
• These beams may be delivered from outside the body (external beam radiation therapy) or the radioactive material maybe placed inside the vagina (internal radiation therapy or brachytherapy)
• Possible side effects may include:
  • Fatigue, nausea, vomiting, and diarrhea
  • Bladder irritation, leading to inflammation (cystitis)
  • Ovaries may be affected resulting in menstrual changes, or premature menopause
  • The vulva and vagina may be affected, causing soreness, or even scar tissue formation

Currently, clinical trials on adjuvant chemotherapy and combinational chemotherapy, as secondary treatment to surgery, 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 Vagina be Prevented?

• There are currently no known methods of preventing Leiomyosarcoma of Vagina
• 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 Vagina? (Outcomes/Resolutions)

• According to the US National Cancer Institute (NCI) database, the 5-year survival rate for vaginal cancers range from 84% for stage I cancers, to 57% for stage IV cancers
• The prognosis for Leiomyosarcoma of Vagina 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. However, typically it is difficult to treat leiomyosarcomas
• Some study reports indicate that the 5 year survival rate may range between 36-43% for women with Vaginal Leiomyosarcomas. The long-term survival is linked to an individual’s response to treatment, particularly exenteration, which has showed better outcomes
• 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 Vagina:

• 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