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

• Extrauterine Leiomyoma - Vulva
• Vulva Leiomyoma

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

• Leiomyoma of Vulva is a rare, benign mass that is present in the vulva (the external opening of the vagina). A leiomyoma is a benign smooth muscle tumor that can develop anywhere in the body
• Just as uterine leiomyomas (or uterine fibroids) are very common tumors of the uterine corpus, Vulvar Leiomyomas are reported to be common mesenchymal tumors affecting the vulva
• The mesenchyme is the middle layer of the 3 primary germ layers of an embryo, namely the ectoderm, mesoderm, and endoderm. The mesoderm gives rise to mesenchymal tissue, which is the source for bone, muscle, connective tissue, and dermis of skin
• Leiomyomas of Vulva are typically painless, solitary, and are observed to be well-circumscribed (in most cases). They are generally found in young and middle-aged women and are influenced by hormonal factors
• Vulvar Leiomyomas may cause abdominal pain, urination difficulties, and pain during sex. Some tumors are known to develop in women after hysterectomy (surgical removal of the uterus) too
• The treatment of choice is a surgical removal of the entire tumor. The prognosis is excellent with appropriate treatment, since Leiomyoma of Vulva is a benign tumor

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

• Leiomyoma of Vulva may affect females of any age, but is mostly present in the 30-60 years age range (young, middle, and elderly women)
• There is no geographical, racial, or ethnic preference noticed

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

The risk factors for Leiomyoma of Vulva may include:

• Family history of the condition
• Imbalance of estrogen and progesterone hormone levels in the body
• Early onset of menstruation (in girls)
• Obesity, being overweight
• High in meat and low in vegetables diet
• Vitamin D deficiency
• Excessive alcohol consumption

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 Leiomyoma of Vulva? (Etiology)

• The exact cause of Leiomyoma of Vulva development is unknown
• It is believed that similar genetic factors that are responsible for leiomyomas of the uterus may play a role in Vulvar Leiomyoma tumor development
• Research is being performed to understand the causative factors of this rare tumor type

Note: Leiomyoma of Vulva is not a sexually-transmitted disease/condition.

What are the Signs and Symptoms of Leiomyoma of Vulva?

A majority of women do not show any signs and symptoms, when the tumors are generally of a small size. The signs and symptoms of Leiomyoma of Vulva may include:

• Most tumors occur as solitary, solid/firm, and well-circumscribed masses
• They may be found just below the skin surface (subcutaneous locations)
• The site of the tumor is the labia majora on the vulva, and the tumor is commonly mistaken for a bartholin’s cyst (a painless enlargement or swelling of the bartholin gland)
• Itching and redness of the vulvar region may be observed
• Most of the tumors are less than 2-3 cm in size; though, they may grow to much larger sizes (in excess of 15 cm)
• Large tumors can cause pain in the pelvic region. They may also cause the following:
  • Feeling of fullness in the abdomen
  • Frequent urination due to compression/pressure of the tumor
  • Lower back pain
  • Pain during sexual intercourse

Leiomyomatosis of vulva: Some women with Vulvar Leiomyomas are known to simultaneously have these smooth muscle tumors (or leiomyomas) elsewhere in the body too, such as in the esophagus (food pipe). This is known as leiomyomatosis of vulva.

How is Leiomyoma of Vulva Diagnosed?

The diagnosis of Leiomyoma of Vulva may involve the following tests and exams:

• 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

Note: 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 Leiomyoma of Vulva?

The possible complications of Leiomyoma of Vulva include:

• Stress and anxiety due to fear of cancer in the vulva
• There may be cosmetic issues due to the presence of a visible tumor
• Post-surgical infection at the wound site is a potential complication
• Incompletely removed/excised large tumors are known to recur (sometimes after a long time duration)
• In a pregnant woman:
  • Leiomyomas can grow to large sizes during pregnancy due to hormonal influence
  • Some tumors may recur during pregnancy

How is Leiomyoma of Vulva Treated?

Following are the treatment methods for Leiomyoma of Vulva:

• Asymptomatic leiomyomas may not require any treatment; in such cases, the healthcare provider may chose to periodically observe and monitor the tumor
• In some individuals, the size of the tumor may shrink after menopause without any treatment
• Medical treatment options:
  • Pain medications
  • Hormonal treatment such as birth control pills
  • Dietary and lifestyle modification to address overweight issues
  • Taking supplements in case of vitamin D deficiency
  • Avoiding alcohol during pregnancy and limiting consumption
• 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
• Surgical treatment options: A simple surgical excision and removal of the entire tumor is normally sufficient treatment
  • Myolysis of leiomyoma: 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
• 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

How can Leiomyoma of Vulva be Prevented?

Current medical research has not established a method of preventing Leiomyoma of Vulva. However, the following factors may be considered to reduce the risk for the tumor development:

• Address any condition causing hormonal imbalance in the body
• Maintain weight through proper diet modification and physical exercises, if you are overweight/obese
• Avoid alcohol consumption or limit its intake
• Have a balanced diet that is not high in meat and low in vegetables; a balanced diet can also help avoid any mineral or vitamin deficiencies in the body
• Regular prenatal checkups are necessary to monitor the health of the expectant mother and baby in the womb

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

• The prognosis of Leiomyoma of Vulva is generally excellent on surgical excision and removal of the tumor, in a majority of cases
• Pregnant women with Vulvar Leiomyomas may have a higher risk for complications, which may affect both the mother and baby
• However, periodic follow-up check-ups with regular screening may be required

Additional and Relevant Useful Information for Leiomyoma of Vulva:

• Leiomyomas may also occur on the male genitalia, commonly on the scrotum (bag-like structure housing the testes)
• Fibroid tumor removal (or myomectomy) is the surgical removal of fibroids from the uterus

The following link will help you understand fibroid tumor removal surgical procedure:

http://www.dovemed.com/common-procedures/procedures-surgical/fibroid-tumor-removal/