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

• Genital Rhabdomyoma - Vulva
• Vulvar Rhabdomyoma

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

• Rhabdomyoma of Vulva is an infrequent benign tumor of the connective tissues, showing mature skeletal muscle differentiation (meaning that it resembles skeletal muscle tissue)
• Vulvar Rhabdomyoma is observed in young and middle-aged women. In the female genital tract, rhabdomyomas are more commonly observed in the vagina, and to a lesser extent, in the vulva. The vulva is the area around the external opening/mouth of the vagina
• There are no clearly established risk factors for Rhabdomyoma of Vulva and the cause of tumor formation is also unknown
• The tumors appear as a single small polyp or nodule in the vulva. No significant signs and symptoms or complications are noted
• Treatment course includes close observation of the tumor in asymptomatic cases and surgical management, if necessary. In general, the prognosis of Rhabdomyoma of Vulva is excellent with suitable treatment

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

• Rhabdomyoma of Vulva is a rare tumor that is observed in young and middle-aged adult women, just like other genital rhabdomyomas
• However, vulva is not an uncommon site of the tumor within the female reproductive system. The other locations may include the cervix and vagina (which is the most common site)
• There is no known ethnic or racial preference

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

• No definitive risk factors have been identified for Rhabdomyoma of Vulva

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 Rhabdomyoma of Vulva? (Etiology)

• Currently, the exact cause and mechanism of formation of Rhabdomyoma of Vulva is unknown
• It is thought to occur spontaneously due to certain morphological abnormalities in the tissues

What are the Signs and Symptoms of Rhabdomyoma of Vulva?

The signs and symptoms of Rhabdomyoma of Vulva may include:

• Small tumors usually do not cause any symptoms. But, occasionally they may become painful, if they compress surrounding structure
• Abnormal vaginal bleeding
• Pain during sexual intercourse; bleeding after sex
• The benign tumor may grow at a slow rate and appear as a well-defined nodule or polyp
• These soft tissue tumors are normally solitary in most of the cases; however, multiple tumors may be present
• Some tumors may grow to large sizes and may cause a feeling of fullness in the abdomen; enlargement of lower abdomen may be present
• Pain in the pelvic region
• Frequent urination due to compression/pressure of the tumor
• Lower back pain

How is Rhabdomyoma of Vulva Diagnosed?

A diagnosis of Rhabdomyoma 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 Rhabdomyoma of Vulva?

Significant complications from Rhabdomyoma 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
• Heavy and prolonged menstrual bleeding may result in anemia
• 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

Recurrence of the tumor after surgery (complete excised and removal) is not known to occur.

How is Rhabdomyoma of Vulva Treated?

Treatment measures for Rhabdomyoma 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 rhabdomyoma
• Pain medications, in case of tumors causing pain
• Treating iron-deficiency anemia, if any
• 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:
  • 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 Rhabdomyoma of Vulva be Prevented?

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

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

The prognosis of Rhabdomyoma of Vulva is excellent with surgical intervention and complete removal, since it is a benign tumor.

Additional and Relevant Useful Information for Rhabdomyoma of Vulva:

• Rhabdomyoma is a benign tumor of the connective tissues, showing skeletal muscle origin. Based on their location, they are generally classified as cardiac and extracardiac types
• Rhabdomyoma of Vulva is an extracardiac type of tumor