Carcinosarcoma of Uterine Corpus

Carcinosarcoma of Uterine Corpus

Article
Women's Health
Diseases & Conditions
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Contributed byMaulik P. Purohit MD MPHAug 12, 2018

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

  • Carcinosarcoma of Corpus Uteri
  • Malignant Mixed Müllerian Tumor of Uterus
  • Uterine Carcinosarcoma

What is Carcinosarcoma of Uterine Corpus? (Definition/Background Information)

  • Carcinosarcoma of Uterine Corpus is a rare, high-grade cancer affecting older women following menopause. It is classified as a mixed epithelial and mesenchymal tumor of the uterine corpus. This cancer type is also known as Malignant Mixed Müllerian Tumor (MMMT) of Uterus
  • The risk factors for tumor development in the uterus include long duration estrogen therapy, radiation treatment to the pelvis, and any condition that affects the hormonal balance in women. The cause of Carcinosarcoma of Uterine Corpus remains unknown, although studies indicate that there is a similarity to endometrial cancer at the genetic level
  • Uterine Carcinosarcomas are generally painless and asymptomatic during the early stages. Symptomatic women are commonly known to have vaginal bleeding and sensation of mass in the pelvic region. Large tumors sizes can cause signs and symptoms due to compression of adjoining organ structures
  • The treatment of choice for Carcinosarcoma of Uterine Corpus is a surgical removal of the entire tumor. However, other treatment modalities, such as radiation and chemotherapy, may also be necessary
  • The prognosis of Carcinosarcoma of Uterine Corpus depends upon a variety of factors that include the stage of the tumor, the age of the individual, and severity of the signs and symptoms. Nevertheless, the prognosis of this aggressive cancer, in many cases, is poor

Who gets Carcinosarcoma of Uterine Corpus? (Age and Sex Distribution)

  • Carcinosarcoma of Uterine Corpus is a very uncommon cancer; around 5% of malignant uterine tumors are carcinosarcomas
  • The tumor is mostly observed in women older than 48-50 years (postmenopausal women), although it may affect females of a wider age range
  • There is no geographical, racial, or ethnic preference noticed

What are the Risk Factors for Carcinosarcoma of Uterine Corpus? (Predisposing Factors)

The specific risk factors for Carcinosarcoma of Uterine Corpus are unknown. However, in general, the risk factors for cancer of the uterus include the following:

  • Women following menopause have the highest risk; the older the women, the greater is the risk
  • Prior history of radiation therapy to the pelvic region: The cancer can occur secondary to radiation therapy to the pelvic region, after many years (may be 10-20 years later)
  • Estrogen therapy for a long time duration
  • Polycystic ovarian syndrome: An endocrine disorder occurring in women of reproductive age
  • History of breast cancer or ovarian cancer
  • Tamoxifen therapy for breast cancer
  • Any other condition causing an hormonal imbalance in the body, such as use of birth-control pills or pregnancy
  • Menstrual cycle: Women who got their period before the age of 12 years, and those who reached menopause after age 55 have a higher risk. The longer the duration between menarche and menopause, the greater is the risk. This is due to hormonal influences during the reproductive period on the breast tissue
  • Obesity or overweight individuals have a higher risk
  • Use of intrauterine contraceptive devices (IUDs)
  • Poorly-controlled diabetes
  • Unhealthy diet and sedentary lifestyles
  • Hereditary nonpolyposis colorectal cancer (HNPCC): This condition is known to increase the risk for cancers affecting the endometrium

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 Carcinosarcoma of Uterine Corpus? (Etiology)

  • The exact cause of Carcinosarcoma of Uterine Corpus development is unknown
  • Genetic studies show similarity to endometrial cancers; many gene mutations and other genetic abnormalities have been identified
  • Research is being performed to understand the causative factors of this high-grade cancer

What are the Signs and Symptoms of Carcinosarcoma of Uterine Corpus?

The signs and symptoms of Carcinosarcoma of Uterine Corpus may include the following:

  • The tumor arises in the body of the uterus (or uterine corpus)
  • Small-sized tumors are generally painless, although larger tumors may cause pelvic and abdominal pain
  • At the time of detection, generally large tumors are observed, which can cause enlargement of the pelvic region
  • Carcinosarcomas are soft tumors that appear like polyps or appear as a mass
  • Some tumors fill the entire uterine space and bulge out of the cervix. Individuals may have a sensation of a mass prolapsing into the cervix (observed in 50% of the cases). In some cases, the tumor may even block the uterine cavity
  • Some tumors show areas of bleeding and tissue death (necrosis)
  • Females experience abnormal vaginal bleeding, which is very common. There may also be abnormal discharge from the vagina
  • Large tumors may compress adjoining organs and structures. This may lead to increased urination frequency
  • Most often involvement of the deep myometrium (and cervix in some cases) is noted
  • The tumors can also invade the lymphatic and vascular systems
  • Associated weight loss, weakness, and lethargy
  • Some women may not experience any symptoms during the initial stages of tumor growth

How is Carcinosarcoma of Uterine Corpus Diagnosed?

The diagnosis of Carcinosarcoma of Uterine Corpus may involve the following tests and procedures:

  • 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
  • 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
  • Differential diagnosis, to eliminate other tumor types is considered, before arriving at a definitive diagnosis

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 Carcinosarcoma of Uterine Corpus?

The possible complications of Carcinosarcoma of Uterine Corpus include:

  • Stress and anxiety due to fear of cancer of the uterus
  • Polypoid tumors may undergo mechanical injury, such as torsion or twisting, which can result in excruciating pain
  • The polyps may cause cervical prolapse
  • Metastasis: In about 35% of the cases, it has been observed that at the time of diagnosis, the tumor has spread to other parts of the body (extra-uterine locations)
  • Local recurrences following surgical removal of the tumor; recurrence is commonly noted in the abdominal or pelvic regions
  • There may be severe complications due to the tumor in pregnant women

How is Carcinosarcoma of Uterine Corpus Treated?

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

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

Stage I uterine cancer: The cancer is found in the uterus only. Stage I is divided into stages IA and IB, based on how far the cancer has spread.

  • Stage IA: The cancer is in the endometrium only or less than halfway through the myometrium (muscle layer of the uterus).
  • Stage IB: The cancer has spread halfway or more into the myometrium.

Stage II uterine cancer: The cancer has spread into connective tissue of the cervix, but has not spread outside the uterus.

Stage III uterine cancer: The cancer has spread beyond the uterus and cervix, but has not spread beyond the pelvis. Stage III is divided into stages IIIA, IIIB, and IIIC, based on how far the cancer has spread within the pelvis.

  • Stage IIIA: The cancer has spread to the outer layer of the uterus and/or to the fallopian tubes, ovaries, and ligaments of the uterus.
  • Stage IIIB: The cancer has spread to the vagina and/or to the parametrium (connective tissue and fat around the uterus).
  • Stage IIIC: The cancer has spread to lymph nodes in the pelvis and/or around the aorta (largest artery in the body, which carries blood away from the heart).

Stage IV uterine cancer: The cancer has spread beyond the pelvis. Stage IV is divided into stages IVA and IVB, based on how far the cancer has spread.

  • Stage IVA: The cancer has spread to the bladder and/or bowel wall.
  • Stage IVB: The cancer has spread to other parts of the body beyond the pelvis, including the abdomen and/or lymph nodes in the groin.

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

In general, Uterine Carcinosarcoma is a highly-aggressive cancer and the treatment can be challenging. A multidisciplinary approach with a team of healthcare specialists and experts from various medical fields may be necessary to treat the cancer at a healthcare institution that has the experience and expertise in dealing with such high-grade cancers.

The treatment of Carcinosarcoma of Uterine Corpus may depend upon a consideration of the following set of factors:

  • Size of the tumor
  • Severity of the signs and symptoms
  • Location of the carcinosarcoma within the uterus
  • Age of the individual
  • Whether the women is pregnant

The following treatment methods may be employed:

  • Surgical treatment options:
    • Myomectomy: Removal of the tumor
    • Hysterectomy: The removal of a part of the uterus or the entire uterus
    • Radical hysterectomy: It involves the removal of the uterus and tissues surrounding it. This procedure is typically more suited for tumors that have spread beyond the uterus
    • Myolysis: In this procedure, a needle is inserted into the tumor. After the insertion, the tumor is destroyed either by using an electric current, or by a freezing technique
    • Tumor embolization is a possible treatment option. Here the blood supply to the tumor is blocked resulting in its death
    • Radiofrequency ablation: In this technique, the tumors are destroyed using radio waves
  • Radiation therapy and chemotherapy are employed depending upon each specific case
  • Clinical trials on adjuvant chemotherapy and combinational chemotherapy, as secondary treatment to hysterectomy, may be helpful
  • Post-operative care is important: One must maintain minimum activity levels, until the surgical wound heals
  • Follow-up care with regular screening and check-ups are important

How can Carcinosarcoma of Uterine Corpus be Prevented?

Currently, it is not possible to prevent Carcinosarcoma of Uterine Corpus. However, controlling certain factors may help lower one’s risk for uterine cancers.

  • Maintain a healthy weight and exercise regularly; physical activity can reduce risk, especially in post-menopausal women
  • Implement and follow a well-balanced diet; a high intake of fiber via fresh fruits and vegetables can reduce the risk
  • Avoiding smoking
  • Using appropriate protective gear while working with x-rays and other radioactive source
  • In order to avoid a relapse, or be prepared for a recurrence, the entire diagnosis, treatment process, drugs administered, etc. should be well-documented and follow-up measures initiated

Regular medical screening at periodic intervals with blood tests, scans, and physical examinations, are mandatory. Often several years of active vigilance are crucial and necessary.

What is the Prognosis of Carcinosarcoma of Uterine Corpus? (Outcomes/Resolutions)

  • Carcinosarcoma of Uterine Corpus is a high-grade malignancy with an extremely aggressive growth pattern. In general, the prognosis of the condition is poor; death commonly occurs either due to tumor recurrence or spread of the tumor (metastasis) to other body sites
  • Study reports indicate that in a majority of cases, during the time of detection, metastasis has already taken place. The spread of the cancer usually involves the lymph nodes of the pelvis and para-aortic region. In some cases, the tumor metastasizes to the lung, bone, and brain. Even at stage I, the prognosis is guarded, especially with the presence of certain carcinomatous elements
  • Research indicates that a significant prognostic indicator is the depth of invasion of the tumor into the myometrium - the deeper it is, the more worse is the prognosis; and, higher are the chances of rapid tumor progression and advanced stage diagnosis
  • In general, the prognosis of the tumor depends upon a set of several factors, which include:
    • Stage of tumor: 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
    • 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
    • The size of the tumor: Individuals with small-sized tumors fare better than those with large-sized tumors
    • Individuals with bulky disease have a poorer prognosis
    • Involvement of vital organs may complicate the condition
    • The surgical respectability of the tumor (meaning, if the tumor can be removed completely) - it is a treatment option
    • 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 (progressive Uterine Carcinosarcoma)

Additional and Relevant Useful Information for Carcinosarcoma of Uterine Corpus:

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.

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Maulik P. Purohit MD MPH picture
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Maulik P. Purohit MD MPH

Assistant Medical Director, Medical Editorial Board, DoveMed Team

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