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

• Primary Cervical Schwannoma
• Primary Schwann Cell Tumor of Uterine Cervix
• Primary Schwannoma of Cervix

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

• Schwannomas are a group of tumors that arise from the Schwann cells around the nerves; these tumors are essentially nerve sheath tumors
• Primary Schwannoma of Uterine Cervix is a very uncommon tumor that forms in the uterine cervix of generally young, middle-aged, and slightly older women
• Currently, no causative factors for Primary Schwannoma of Uterine Cervix have been identified. In case of the presence of multiple schwannomas (in a minority of cases), the risk factors for the tumor include the presence of neurofibromatosis type 2 (NF2) and a positive family history of schwannoma
• Primary Schwannoma of Uterine Cervix can cause signs and symptoms that include severe pelvic pain, abnormal vaginal bleeding, and urination difficulties (if the tumors are large). In rare cases, the schwannomas are known to develop into malignant tumors. The tumors can also compress the nerve on which it lies and cause nerve dysfunction
• In a majority of cases, a complete excision by surgery can be curative and the prognosis of Primary Schwannoma of Uterine Cervix is excellent. However, the prognosis may be influenced by factors that include the subtype of schwannoma, tumor size, the presence of NF2, and if any malignant transformations are noted

Based upon the clinical behavior, genetic mutations, and tissue biopsy studies, schwannomas can be subdivided into the following types:

• Benign conventional schwannoma: Conventional schwannomas are typically benign; though rarely, they can become malignant
• Melanotic schwannoma: A tumor of low malignancy potential

There are various histological types of schwannomas, which include:

• Cellular schwannoma: This is a type of schwannoma which has characteristic features, when examined under a microscope by a pathologist
• Plexiform schwannoma: This type of schwannoma affects multiple nerves; it can also affect the nerve plexus (group of nerves). Only a minority of individual with this type are known to have a history of neurofibromatosis type 2 (NF2)
• Microcystic (reticular) schwannoma: It is a very uncommon type of schwannoma having a characteristic microscopic appearance

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

• From the cases that have been studied, Primary Schwannoma of Uterine Cervix is seen to occur within the age range of 25-70 years, though generally middle-aged women are affected the most
• In general, when schwannomas occur against a background of neurofibromatosis type 2 (NF2), they are seen before the age of 30 years
• Schwannomas have no known geographical, racial, or ethnic preference; they are seen worldwide

What are the Risk Factors for Primary Schwannoma of Uterine Cervix? (Predisposing Factors)

The specific risk factors for Primary Schwannoma of Uterine Cervix are unknown or unidentified. However, generally the following factors may be observed with respect to schwannomas:

• 9 in 10 cases of schwannoma are solitary in nature with generally no identifiable risk factors
• On the remaining cases, there may be the presence of multiple tumors in the body, in which case the risk factors may include:
  • Neurofibromatosis type 2 (NF2), a genetic condition that is characterized by the formation of non-cancerous tumors that affect the nervous system. Approximately 4% of the schwannomas are associated with NF2
  • A family history of the tumor
  • Schwannomatosis: It is a genetic condition that is a usually seen in adults and manifests as multiple schwannomas
  • Gorlin-Koutlas syndrome: A complex genetic disorder of multiple tumors in the body including multiple schwannomas
  • Trauma: It is often associated with plexiform schwannomas

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 Primary Schwannoma of Uterine Cervix? (Etiology)

The exact cause and mechanism of Primary Schwannoma of Uterine Cervix formation, in a majority of cases, is unknown. Solitary tumors are believed to be the result of sporadic mutations, implying that they do not have a preceding family history of the condition.

• Multiple schwannomas are known to occur in a background of genetic disorders, such as neurofibromatosis type 2 (NF2), Gorlin-Koutlas syndrome, or a positive family history
• In general, the tumor is believed to be caused by the overproduction of Schwann cells that wrap around the nerve

What are the Signs and Symptoms of Primary Schwannoma of Uterine Cervix?

In general, schwannomas are slow-growing tumors and a majority does not exhibit any significant signs and symptoms. They are typically detected incidentally, when the individual is being examined for some other medical condition.

The signs and symptoms of Primary Schwannoma of Uterine Cervix may include:

• Most tumors occur as solitary, well-circumscribed masses; however, the presence of multiple tumors at various locations of the female genital system may be noted
• Pain in the pelvic region; the pain may be severe
• Abnormal vagina bleeding or discharge
• Some tumors may grow to large sizes and may cause a feeling of fullness in the abdomen; enlargement of lower abdomen
• Frequent urination due to compression/pressure of the tumor
• Lower back pain
• Pain during sexual intercourse

How is Primary Schwannoma of Uterine Cervix Diagnosed?

A diagnosis of Primary Schwannoma of Uterine Cervix is made using the following tools:

• Complete physical (pelvic) 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
• Hysteroscopy: This procedure involves placing a probe through the cervix to examine the cavity of the uterus. This exam is helpful in detecting submucosal leiomyomas
• 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 Primary Schwannoma of Uterine Cervix?

The complications from Primary Schwannoma of Uterine Cervix could include:

• Stress and anxiety due to fear of cancer in the cervix
• Heavy and prolonged menstrual bleeding may result in anemia
• Fertility issues due to mechanical obstruction of the cervical opening, if tumors are large
• Compression of the underlying nerve, which can severely affect the nerve function
• Damage to the nerve during surgery to remove the tumor. Sometimes, removing the tumor can worsen the signs and symptoms in some individuals
• Infrequently, recurrence of the tumor after surgery, if the tumor is incompletely removed
• Very rarely, schwannomas can become malignant (malignant transformation of schwannoma); 1% of tumors are observed to become cancerous
• Post-surgical infection at the wound site is a potential complication

How is Primary Schwannoma of Uterine Cervix Treated?

In many cases, if Primary Schwannoma of Uterine Cervix is solitary, small-sized, and there are no significant symptoms, then no treatment may be necessary. Periodic observations can be maintained through regular check-ups.

Surgical excision and tumor removal is the usual mode of treatment of schwannoma. A very important criterion for surgery is to preserve nerve function that is affected by the tumor. Surgical treatment options may include the following:

• Pain medications, in case of tumors causing pain
• Treating iron-deficiency anemia, if any
• In pregnant women:
  • Once the condition is diagnosed, the pregnant mother is closely monitored
  • The healthcare provider may recommend an increased frequency of prenatal appointments to monitor progress of the baby’s growth
  • A cesarean section (C-section) delivery of the baby may be necessary, in some cases
• Surgical intervention with complete excision can result in a complete cure. It can also help reduce the chances of tumor recurrence
  • Myomectomy: Removal of the tumor/fibroid (also known as fibroidectomy)
  • Myolysis of the tumor: 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 tumor death
  • Radiofrequency ablation: In this technique, the tumors are destroyed using radio waves
• Radiation therapy and chemotherapy are not usually required (if the tumors are benign)

The surgery may be performed through any of the following techniques:

• Microsurgery: The surgery is performed using microsurgical techniques, which can help minimize damage to the accompanying nerve. Since the tumor mostly affects the covering around the nerves, it can be completely removed without affecting the nerve in most cases. The following techniques may be employed:
  • Subtotal removal; when the tumor is incompletely removed
  • Near total removal; when most of the tumor is removed and there is very little tumor remnants
  • Total tumor removal; when the tumor is removed completely
• Stereotactic radiosurgery is a relatively new treatment method that can be helpful in some individuals. It is a radiation therapy technique and not an invasive surgical procedure. It can be administered using a Gamma Knife (Gamma Knife radiosurgery)

In case of malignancy, additional treatment options, such as chemotherapy and/or radiation therapy, may be considered.

• Chemotherapy may be used to destroy the tumor cells
• Radiation therapy may be useful in individuals who are not candidates for surgery. Such cases include:
  • Individuals with overall poor health status
  • Individuals with minimal symptoms
• Treatment of the underlying neurofibromatosis type 2, if present
• 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 Primary Schwannoma of Uterine Cervix be Prevented?

Current medical research has not established a way of preventing Primary Schwannoma of Uterine Cervix. However, in case it is associated with genetic disorders, such as neurofibromatosis type 2, then the following may be considered:

• Genetic testing of the expecting parents (and related family members) and prenatal diagnosis (molecular testing of the fetus during pregnancy) may help in understanding the risks better during pregnancy
• If there is a family history of the condition, then genetic counseling will help assess risks before planning for a child
• Active research is currently being performed to explore the possibilities for treatment and prevention of inherited and acquired genetic disorders such as neurofibromatosis type 2
• Regular medical screening at periodic intervals with tests and physical examinations are strongly recommended

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

• The prognosis of Primary Schwannoma of Uterine Cervix (benign tumor) is excellent, if the tumor can be removed without damaging the underlying nerve. Most schwannomas are benign and do not recur when completely excised
• However, this also depends upon the subtype of the tumor, the tumor size, and the presence of neurofibromatosis type 2. If benign schwannomas transform to malignant schwannomas (in very rare occasions), then the prognosis may depend upon a set of several factors including the stage of the tumor, its location, overall health of the individual, and response to therapy
• Asymptomatic tumors may require no treatment; unless they cause discomfort, affect the quality of life, or affect functioning of the underlying nerve. In such cases, close monitoring of the Primary Cervical Schwannoma is a treatment option

Additional and Relevant Useful Information for Primary Schwannoma of Uterine Cervix:

A differential diagnosis may have to be undertaken to eliminate other tumors such as:

• Neurofibroma
• Leiomyosarcoma
• Gastrointestinal stromal tumor (GIST)
• Malignant peripheral nerve sheath tumor (MPNST)

Please visit our Cancer & Benign Tumor Health Center for more physician-approved health information:

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