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

• Superficial MFB of Vulva
• Vulvar Superficial MFB
• Vulvovaginal Superficial Myofibroblastoma

What is Myofibroblastoma of Vulva? (Definition/Background Information)

• Superficial Myofibroblastoma (MFB) of Vulva can be described as an unusual, benign, soft tissue tumor affecting both the vulva and vagina (vulvovaginal region). This tumor of the female genital tract is generally observed in adult women
• The cause of Superficial Myofibroblastoma of Vulva is unknown, but the risk factors may include hormone therapy and tamoxifen therapy
• The signs and symptoms of Superficial Myofibroblastoma of Vulva may include the presence of a slow-growing lump in the vulva (area around the external opening of the vagina). Small-sized tumors may be asymptomatic and show no signs and symptoms, while large tumors are present as a mass
• Superficial Myofibroblastoma of Vulva is treated through a surgical excision, per the healthcare provider’s recommendation, which is curative
• The prognosis is excellent with its complete removal, since it is a benign tumor. However, periodic checkups and follow-up visits are advised

Who gets Myofibroblastoma of Vulva? (Age and Sex Distribution)

• Superficial Myofibroblastoma of Vulva is a rare and benign tumor that can affect young, middle-aged, and older adult women in the 20-80 year age category
• All racial and ethnic groups are affected and no specific predilection is seen

What are the Risk Factors for Myofibroblastoma of Vulva? (Predisposing Factors)

The specific risk factors for Superficial Myofibroblastoma of Vulva are unknown or unidentified.

• Some association of the tumor with therapy using tamoxifen drug has been noted
• Hormone replacement therapy in some women may be a risk factor

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 Myofibroblastoma of Vulva? (Etiology)

• The exact cause of development of Superficial Myofibroblastoma of Vulva is currently not clearly understood
• Certain chromosomal aberrations have also been reported in the tumors
• Research has established that such genetic anomalies are similar to those seen in cellular angiofibromas, spindle cell lipomas, and myofibroblastomas of breast

What are the Signs and Symptoms of Myofibroblastoma of Vulva?

The signs and symptoms of Superficial Myofibroblastoma of Vulva may include:

• Presence of a solitary benign lump in the vulva or vulvovaginal region
• The tumor is a slow-growing mass
• The tumors can appear as a well-defined and firm mass during an imaging study; the lesion may be nodular or polypoid (like a polyp)
• The tumor size may range from a few mm to nearly 5 cm
• The tumor is generally painless, but large sizes may cause discomfort while walking, during sex

In case the tumor involves both the vagina and uterine cervix, then it is known as a ‘superficial cervicovaginal myofibroblastoma’.

How is Myofibroblastoma of Vulva Diagnosed?

The following diagnostic tools may be used to diagnose Superficial Myofibroblastoma of Vulva:

• 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
• Colposcopy:
  • The cervix (including the vagina and vulva) 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

Although the above modalities can be used to make an initial diagnosis, a tissue biopsy of the tumor is necessary to make a definitive diagnosis to begin treatment. The tissue for diagnosis can be procured in multiple different ways which include:

• Fine needle aspiration (FNA) biopsy of the tumor: A FNA biopsy may not be helpful, because one may not be able to visualize the different morphological areas of the tumor. Hence, a FNA biopsy as a diagnostic tool has certain limitations, and an open surgical biopsy is preferred
• Core biopsy of the tumor
• Open biopsy of the tumor

Tissue biopsy:

• A tissue biopsy of the tumor is performed and sent to a laboratory for a pathological examination. A pathologist 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. Examination of the biopsy under a microscope by a pathologist is considered to be gold standard in arriving at a conclusive diagnosis
• Biopsy specimens are studied initially using Hematoxylin and Eosin staining. The pathologist then decides on additional studies depending on the clinical situation
• Sometimes, the pathologist may perform special studies, which may include immunohistochemical stains, molecular testing, and very rarely, electron microscopic studies to assist in the 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 Myofibroblastoma of Vulva?

There may not be any significant complications of Superficial Myofibroblastoma of Vulva.

• However, in some cases, the condition may cause emotional stress due to concerns of a malignancy
• Severe pain during sex
• Damage to the muscles, vital nerves, and blood vessels, during surgery
• Post-surgical infection at the wound site is a potential complication

How is Myofibroblastoma of Vulva Treated?

The following treatment methods for Superficial Myofibroblastoma of Vulva may be considered:

• A ‘wait and watch’ approach may be considered for asymptomatic tumors, once a diagnosis of superficial myofibroblastoma has been established
• Surgical intervention with complete excision can result in a complete cure. It can also help reduce the chances of tumor recurrence
• Radiation therapy and chemotherapy are not usually required
• Follow-up care with regular screening may be recommended by the healthcare provider

How can Myofibroblastoma of Vulva be Prevented?

• The development of myofibroblastoma is difficult to prevent. Currently, no specific preventive measures are available to avoid Vulvar Superficial Myofibroblastomas
• Limit combination hormone therapy used to treat symptoms of menopause. It is advised that individuals be aware of the potential benefits and risks of hormone therapy
• Medical screening at regular intervals with scans and physical examinations are advised to detect any early recurrences

What is the Prognosis of Myofibroblastoma of Vulva? (Outcomes/Resolutions)

• The prognosis of Superficial Myofibroblastoma of Vulva is generally excellent on a surgical excision and removal of the tumor, since myofibroblastomas are typically benign
• Periodic follow-up check-ups with screening may be required

Additional and Relevant Useful Information for Myofibroblastoma of Vulva:

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/