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

• Cervical Malignant Peripheral Nerve Sheath Tumor
• Malignant Peripheral Nerve Sheath Tumor of Cervix
• MPNST of Uterine Cervix

What is Malignant Peripheral Nerve Sheath Tumor of Uterine Cervix? (Definition/Background Information)

• Malignant peripheral nerve sheath tumor (MPNST) is a type of soft tissue sarcoma (a malignant type of tumor). Malignant Peripheral Nerve Sheath Tumor of Uterine Cervix is an extremely rare tumor observed in women
• Typically, in about 50% of the cases, the tumors occur against a background of neurofibromatosis type 1 (NF1, a genetic disorder affecting the skin and other body systems). However, based on the few reported cases of Cervical Malignant Peripheral Nerve Sheath Tumor, the involvement of NF1 has not been observed
• These cervical tumors may result in abnormal vaginal bleeding or put pressure on adjoining organs, if they grow to large sizes. Malignant Peripheral Nerve Sheath Tumors of Uterine Cervix are regarded as a kind of malignant tumor that spreads and infiltrates the surrounding tissues, tendons, muscles, and organs
• A combination of chemotherapy, radiation therapy, and surgical procedures, are used to treat Malignant Peripheral Nerve Sheath Tumor of Uterine Cervix. The outcome for an individual with this tumor depends on several factors including the stage of the tumor and overall health status of the individual

In general, there are 2 major types of malignant peripheral nerve sheath tumors:

• Spindled malignant peripheral nerve sheath tumor (95% of the cases are of this type)
• Epithelioid malignant peripheral nerve sheath tumor (constitute about 5% of the cases)

Such classification has not been described in the uterine cervix, because of the rarity of cases.

Who gets Malignant Peripheral Nerve Sheath Tumor of Uterine Cervix? (Age and Sex Distribution)

• Malignant Peripheral Nerve Sheath Tumor of Uterine Cervix is a very rare tumor with only about 10-15 cases being reported in the medical literature
• The tumor may be seen in young and middle-aged women (20-60 years), but most cases are seen in older women above 50 years
• There is no known racial, ethnic, or geographical preference observed

What are the Risk Factors for Malignant Peripheral Nerve Sheath Tumor of Uterine Cervix? (Predisposing Factors)

• Currently, no specific risk factors for the formation of Malignant Peripheral Nerve Sheath Tumor of Cervix have been defined
• However, in general, around 50% of MPNST (seen at other body locations) are associated with neurofibromatosis type 1 (NF1). NF1 is a genetic condition that is characterized by skin pigmentation and the formation of non-cancerous tumors that affect the central nervous system

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 Malignant Peripheral Nerve Sheath Tumor of Uterine Cervix? (Etiology)

• The exact cause and mechanism of formation of Malignant Peripheral Nerve Sheath Tumor of Uterine Cervix is unknown
• Generally, the tumors are thought to occur due to chromosomal defects occurring on account of certain genetic mutations

From the few cases that have been studied, MPNST of Uterine Cervix is not known to be associated with neurofibromatosis type 1.

What are the Signs and Symptoms of Malignant Peripheral Nerve Sheath Tumor of Uterine Cervix?

The signs and symptoms of Malignant Peripheral Nerve Sheath Tumor of Uterine Cervix may include:

• The presence of a large mass in the cervix; the tumor may also appear as a polyp (polypoid)
• The tumors may grow up to 6 cm in size
• Abnormal or irregular bleeding
• Large tumors may put pressure on the surrounding structures and organs and lead to associated signs and symptoms such as enlargement of lower abdomen
• Pain in the pelvic region
• Frequent urination due to compression/pressure of the tumor
• Lower back pain
• Pain during sexual intercourse

How is Malignant Peripheral Nerve Sheath Tumor of Uterine Cervix Diagnosed?

A diagnosis of Malignant Peripheral Nerve Sheath Tumor of Uterine Cervix may involve the following steps:

• 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:

• A tissue biopsy cannot help definitively diagnose a benign MPNST from a malignant MPNST. A tissue biopsy may show overlapping features between a malignant peripheral nerve sheath tumor and a benign peripheral nerve sheath tumor, when examined under a microscope by a pathologist. Clinical correlation as regards the behavior of the tumor is often necessary for a definitive diagnosis of malignancy
• Pap smear is not a good screening tool for Cervical MPNST

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 Malignant Peripheral Nerve Sheath Tumor of Uterine Cervix?

The complications of Malignant Peripheral Nerve Sheath Tumor of Uterine Cervix may include:

• Metastasis of the tumor to other body sites, such as to the bones, lungs, or lymph nodes, may occur. Nevertheless, metastasis is observed very infrequently with Cervical MPNST
• Damage of vital nerves, blood vessels, and surrounding structures, during surgery to remove the tumor
• Side effects from chemotherapy (toxicity), radiation therapy
• Sexual dysfunction can take place as a side effect of surgery, chemotherapy, or radiation therapy
• Recurrence of the tumor in case of incomplete excision and removal

How is Malignant Peripheral Nerve Sheath Tumor of Uterine Cervix Treated?

Once a diagnosis of cervical cancer has been made, the extent to which the tumor has spread is assessed, known as 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 Malignant Peripheral Nerve Sheath Tumor of Uterine Cervix may be undertaken as:

• Any combination of chemotherapy, radiation therapy, and invasive procedures may be used to treat Cervical MPNST
• 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
• 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 Malignant Peripheral Nerve Sheath Tumor of Uterine Cervix be Prevented?

• Current medical research have not established a way of preventing Malignant Peripheral Nerve Sheath Tumor of Uterine Cervix
• Regular medical screening at periodic intervals with blood tests, scans, and physical examinations, are mandatory, due to its metastasizing potential and possibility of recurrence. Often several years of active vigilance is necessary

What is the Prognosis of Malignant Peripheral Nerve Sheath Tumor of Uterine Cervix? (Outcomes/Resolutions)

• The prognosis for Malignant Peripheral Nerve Sheath Tumor of Uterine 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. However, Cervical MPNSTs are generally aggressive and many tend to recur following their surgical excision and removal
• Nevertheless, in general, from the reported cases it has been observed that tumors arising in the cervix seem to be less aggressive than tumors arising at other soft tissue locations

Additional and Relevant Useful Information for Malignant Peripheral Nerve Sheath Tumor of Uterine Cervix:

The following DoveMed website links are useful resources for additional information:

http://www.dovemed.com/healthy-living/womens-health/

http://www.dovemed.com/diseases-conditions/cancer/