Alveolar Soft-Part Sarcoma of Uterine Cervix

Alveolar Soft-Part Sarcoma of Uterine Cervix

Article
Sexual Health
Women's Health
+2
Contributed byKrish Tangella MD, MBAApr 13, 2018

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

  • Alveolar Soft-Part Sarcoma of Cervix
  • ASPS of Uterine Cervix
  • Cervical Alveolar Soft-Part Sarcoma

What is Alveolar Soft-Part Sarcoma of Uterine Cervix? (Definition/Background Information)

  • Alveolar Soft Part Sarcoma (ASPS) of Uterine Cervix is an extremely infrequent, but highly malignant soft tissue tumor observed in girls, young adults, and slightly older adult women
  • The tumor is known to form due to chromosomal aberrations (on chromosome 17 and X chromosome) causing abnormal fused genes. This abnormal gene forms a certain kind of protein that is responsible for the tumor formation
  • Most alveolar soft part sarcoma tumors are well-defined and form deep within the body tissues. Cervical ASPS often presents as a solid nodule or polyp within the cervix. The key symptom observed is abnormal vaginal bleeding, which is noted during early stages of cancer formation
  • Due to this, an early detection of Alveolar Soft Part Sarcoma of Cervix is enabled. The treatment involves surgical removal of the tumor, which is usually followed by radiation therapy
  • With adequate treatment of Alveolar Soft Part Sarcoma of Uterine Cervix (surgical excision and tumor removal), the prognosis is generally good. Late detection and older age of the individual may portray a poorer prognosis

Who gets Alveolar Soft-Part Sarcoma of Uterine Cervix? (Age and Sex Distribution)

  • Alveolar Soft Part Sarcomas of Uterine Cervix are extremely rare cancers. Presently, less than 25 cases have been reported in the medical literature
  • Studies inform that less than 1% of all tumors of the cervix are alveolar soft part sarcomas. Even though ASPS of the female genital tract is very uncommon, the cervix is the most common site for this tumor
  • From among the cases observed, ASPS of Cervix is present in a wide age group of females - from young girls to older women (8-60 years age range, average age 32 years) 
  • There is no known ethnic or racial preference observed

What are the Risk Factors for Alveolar Soft-Part Sarcoma of Uterine Cervix? (Predisposing Factors)

  • The risk for Alveolar Soft Part Sarcoma of Uterine Cervix is linked to genetic defects on chromosome 17 and X chromosome

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 Alveolar Soft-Part Sarcoma of Uterine Cervix? (Etiology)

The exact cause and mechanism of Alveolar Soft Part Sarcoma formation of Uterine Cervix is unknown. The tumor is said to arise from embryonic connective tissues.

  • ASPS tumors are thought to occur as a result of gene fusion. The fusion gene occurs when small pieces of two chromosomes break and join to form a new combination gene
  • The genes involved in the process include the ASPL gene on chromosome 17 and the TFE3 gene on chromosome X
  • Due to the creation of this new gene, certain ‘new’ protein forms that is previously not found in healthy cells. This protein is responsible for the formation of alveolar soft part sarcoma
  • However, the exact process of how the tumor forms and develops due to this process is still unclear

What are the Signs and Symptoms of Alveolar Soft-Part Sarcoma of Uterine Cervix?

The signs and symptoms of Alveolar Soft Part Sarcoma of Cervix may include:

  • Abnormal vaginal bleeding is an early indicative sign of tumor presence
  • Presence of a small nodule or polyp in the uterine cervix
  • The size of the tumor can range from 2 mm to 8 cm (mean size of 2.5 cm)
  • ASPS tumors in the cervix are usually well-defined and firm/solid
  • Functional impairment of organs are not observed until advanced stages of the tumor development takes place
  • Pain in the pelvic region from large tumors
  • Frequent urination due to compression/pressure of the tumor
  • Lower back pain
  • Pain during sexual intercourse

Contrary to ASPS tumors at other locations, wherein during the initial growing phase of the tumors, they are normally asymptomatic (making for a difficult early diagnosis); Cervical ASPSs present with early abnormal bleeding, which is a strong indicator for further investigation. Hence, detection of the cancer is often made during the initial stages of its growth and development.

How is Alveolar Soft-Part Sarcoma of Uterine Cervix Diagnosed?

The diagnosis of Alveolar Soft Part Sarcoma of Uterine Cervix may involve the following tests and exams:

  • Evaluation of the individual’s medical history and a thorough physical (pelvic) examination
  • Ultrasound scan of the abdomen
  • CT or CAT scan with contrast of the abdomen and pelvis may show a well-defined mass. This radiological procedure creates detailed 3-dimensional images of structures inside the body
  • MRI scans of the abdomen and pelvis: Magnetic resonance imaging (MRI) uses a magnetic field to create high-quality pictures of certain parts of the body, such as tissues, muscles, nerves, and bones. These high-quality pictures may reveal the presence of the tumor
  • A Pap smear may be ordered as a screening procedure. An abnormal Pap smear warrants further testing
  • Colposcopy: The cervix is examined with an instrument, called a colposcope. This helps the physician get a magnified view of the cervix

Cervical biopsy: Biopsy is the process of removing tissue for examination. A pathologist looks at the tissue sample under a microscope, to detect any evidence of cancer. Types of cervical biopsies include:

  • Colposcopic biopsy: The abnormal areas of the cervix are visualized with a colposcope. After numbing the cervix with a local anesthetic, an instrument, called a biopsy forceps, is used to get a tissue sample.  Mild cramps, pain, and some light bleeding, may occur following the procedure
  • Endocervical curettage (endocervical scraping): The curette is an instrument that can be used to scrape out tissue. Using a curette, cells are scraped out from the endocervix (the inner part of the cervix, close to the uterus/womb) and examined under a microscope. Mild pain and bleeding maybe present following the procedure.
  • Cone biopsy or conization:
    • 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
    • The cone biopsy has the added advantage that it also serves as a treatment for pre-cancers and some early cancers

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

If tumor has metastasized (into the lungs or other regions) then PET scan, bone scan, and chest X-rays may be taken.

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

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 Alveolar Soft-Part Sarcoma of Uterine Cervix?

The complications from Alveolar Soft Part Sarcoma of Uterine Cervix could include:

  • Emotional stress due to the presence of cervical cancer
  • Ulceration of large tumor masses may get secondarily infected with bacteria or fungus
  • A late metastasis of the tumor to other sites, such as to the lungs, brain, spinal cord, and bones, may occur. However, due to its early detection, spread of the cervical tumor to other locations has been observed very infrequently
  • Recurrence of the tumor following its incomplete surgical excision and removal; the recurrence may take place after a long time period
  • Damage to vital nerves, blood vessels, and surrounding structures during surgery
  • Sexual dysfunction can take place as a side effect of surgery, chemotherapy, or radiation therapy
  • Side effects from chemotherapy (such as toxicity) and radiation therapy

How is Alveolar Soft-Part Sarcoma 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.

  • The FIGO (International Federation of Gynecology and Obstetrics) and the AJCC (American Joint Committee on Cancer) TNM staging systems - are two similar, commonly used staging systems
  • A tumor that remains confined only to the superficial layers of the cervix is said to be carcinoma in situ (Stage 0 according to AJCC system)
  • Depending on how far the tumor has spread beyond the cervix, stages I through IV are defined
  • Stage I cancer is confined wholly to the cervix (the cancer has spread to involve even the deeper layers of the cervix unlike stage 0)
  • Stage IV cancer involves the nearby organs, such as the bladder, rectum, or even other distant organs

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)

Any combination of chemotherapy, radiation therapy, and invasive surgical procedures may be used to treat Alveolar Soft Part Sarcoma of Uterine Cervix. However, chemotherapy (either oral drugs or intravenous administration) is usually not found to be very effective.

  • Surgery may include the following procedures:
    • 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
  • Arterial embolization may be used to provide temporary relief from the symptoms, and reduce blood loss during a surgical procedure
  • 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
  • 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 or the uterus (internal radiation therapy or brachytherapy)
  • 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 and encouraged

How can Alveolar Soft-Part Sarcoma of Uterine Cervix be Prevented?

  • Current medical research have not established a way of preventing Alveolar Soft Part Sarcoma of Uterine Cervix
  • The presence of any abnormal vaginal bleeding or discharge should be immediately informed to the physician and periodic follow-up screening maintained
  • Regular medical screening at periodic intervals with blood tests, scans and physical examinations are mandatory for those who have already endured ASPS. This is due to both its high metastasizing potential and chances of recurrence. Often several years of active vigilance is necessary

What is the Prognosis of Alveolar Soft-Part Sarcoma of Uterine Cervix? (Outcomes/Resolutions)

  • Alveolar Soft Part Sarcoma of Uterine Cervix is a rare, but highly-malignant tumor. The long-term prognosis depends on a combination of factors such as:
    • 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
    • FIGO grade of the tumor: Tumors that are graded 1 and 2 have better prognoses than grade 3 tumors
    • Cell growth rate of the cancer
    • Its ki-67 value - a protein found in cells that is a good indicator of how fast the tumor cells are growing. The ki-67 value is determined by a pathologist and is usually mentioned in the pathology report
    • 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
  • Young age of the individual, early detection and small sizes of the tumor are favorable indicators. With complete surgical removal of the tumor, the chances of recurrence are also minimal
  • Large tumor sizes at diagnosis and spread of the tumor to various sites in older individuals can adversely affect the prognosis
  • However, among the few cases recorded, the outcomes were good due to early detection of the tumor (due to the presence of active vaginal bleeding) and its surgical resectability
  • 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 Alveolar Soft-Part Sarcoma of Uterine Cervix:

  • The most common location for alveolar soft part sarcoma is the thigh, leg, and buttock. Other not so common locations include the head and neck (usually in children), female genitalia, and abdominal cavity

Please visit the following link to learn more about alveolar soft part sarcoma:

http://www.dovemed.com/diseases-conditions/alveolar-soft-part-sarcoma-asps/

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Krish Tangella MD, MBA

Pathology, Medical Editorial Board, DoveMed Team

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