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

• Vulvar Neurofibroma

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

• Neurofibroma of Vulva is an infrequent benign tumor that appears as a small bump on the vulvar skin. The vulva is the area around the external opening/mouth of the vagina
• Neurofibroma is a nerve tumor that arises from the nerves underneath the skin surface, or subcutaneously. The risk factors and cause of development of Neurofibroma of Vulva is unknown
• In some cases, the presence of multiple tumors may indicate the presence of neurofibromatosis type 1 (NF-1), while solitary tumors are not associated with this genetic disorder
• The tumors appear as small nodules in the vulva; no significant signs and symptoms may be noted, although large tumors may present severe abdominal/pelvic pain, urination difficulties, and pain during sex
• Treatment course includes close observation of the tumor in asymptomatic cases and surgical management, if necessary. In general, the prognosis of Neurofibroma of Vulva is excellent with suitable treatment

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

• Neurofibroma of Vulva is a rare tumor that is observed in young and middle-aged adult women, just like other genital neurofibromas
• If associated with neurofibromatosis type 1, then the tumor may be present at an early age
• Vulva is the most common site of the tumor within the female genital tract. The other, even rarer  locations may include the vagina, cervix, uterus, and ovaries
• There is no known ethnic or racial preference

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

No definitive risk factors have been identified for solitary Neurofibroma of Vulva. A majority of the solitary tumors occur sporadically.

• However, if more than one tumor is present, then an association with neurofibromatosis type 1 (NF-1) is observed
• NF-1, or von Recklinghausen disease, 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 reported that almost 18% of women with NF-1 may have involvement of the vulva
• A few isolated cases of Vulvar Neurofibromas have been associated with trauma due to surgery

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 Neurofibroma of Vulva? (Etiology)

• Currently, the exact cause and mechanism of formation of Neurofibroma of Vulva is unknown
• It is thought to occur spontaneously due to certain morphological abnormalities in the tissues
• The presence of multiple neurofibromas may indicate the presence of neurofibromatosis type 1, which is an autosomal dominant genetic disorder

What are the Signs and Symptoms of Neurofibroma of Vulva?

The signs and symptoms of Neurofibroma of Vulva may include:

• Small tumors usually do not cause any symptoms; the neurofibroma can occur as tiny bumps just below the skin surface
• The tumors are solid, firm, and mobile (when felt by touch)
• These benign tumors may also be pedunculated (bag-like and hanging from the skin surface); some may grow to very large sizes, in excess of 10-15 cm
• The skin surface around the tumors may be pale tan
• The tumors are normally solitary in most of the cases; however, multiple tumors may be present when associated with neurofibromatosis type 1
• Pain during sexual intercourse
• Severe and chronic pain in the pelvic region
• Frequent urination due to compression/pressure of the tumor
• Lower back pain

How is Neurofibroma of Vulva Diagnosed?

A diagnosis of Neurofibroma of Vulva 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
• Colposcopy:
  • The cervix (including the vulva and vagina) 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 Neurofibroma of Vulva?

Significant complications from Neurofibroma of Vulva are generally not noted, because it is a benign tumor. However, the following may be observed in some cases:

• Stress due to a concern for vulvar cancer
• Cosmetic issues
• Complications may arise due to neurofibromatosis type 1 (if present)
• Severe pain and bleeding during sex may occur in rare cases, if the tumor is ulcerated
• Damage to the muscles, vital nerves, and blood vessels, during surgery
• Post-surgical infection at the wound site is a potential complication
• In some cases, tumor recurrence has been noted

How is Neurofibroma of Vulva Treated?

Treatment measures for Neurofibroma of Vulva may include the following:

• If there are no symptoms, then the healthcare provider may advise a ‘wait and watch’ approach, following a diagnosis of neurofibroma
• Pain medications, in case of tumors causing pain
• Surgical intervention with complete excision can result in a complete cure. It can also help reduce the chances of tumor recurrence. The surgical procedures may include:
  • Vulvectomy: It is a surgical intervention technique to remove a part or the entire vulva
  • 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
• Some women may require reassurance and support to help with stress and anxiety. The healthcare professional may dispel fears that the condition is not due to a sexually-transmitted infection
• Post-operative care is important: Minimum activity level is to be ensured until the surgical wound heals
• Follow-up care with regular screening and check-ups are important

How can Neurofibroma of Vulva be Prevented?

• Current medical research has not established a method of preventing Neurofibroma of Vulva
• Medical screening at regular intervals with scans and physical examinations are advised

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

• The prognosis of Neurofibroma of Vulva is excellent with surgical intervention and complete removal, since it is a benign tumor
• The prognosis of neurofibromas associated with neurofibromatosis type 1, depends upon the severity of the signs and symptoms of the genetic disorder

Additional and Relevant Useful Information for Neurofibroma of Vulva:

Neurofibroma of skin is not an infectious condition and it does not spread from one individual to another.