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

• Vulvar Superficial Angiomyxoma

What is Superficial Angiomyxoma of Vulva? (Definition/Background Information)

• Superficial Angiomyxoma of Vulva is a benign tumor located in the skin and mucosal surface of the vulva (area around the external opening of the vagina) with subcutaneous tissue involvement. It is mostly found in young adult women
• There are no clearly established risk factors for Vulvar Superficial Angiomyxoma and the cause of tumor formation is also unknown
• The tumor appears as a well-defined polyp and is not aggressive, unlike aggressive angiomyxomas that can invade surrounding areas and destroy adjoining structures and organs
• No significant signs and symptoms or complications are typically noted, if the tumors are small-sized. However, in some cases, the tumor is known to recur following an incomplete surgical excision and removal of the angiomyxoma
• The treatment of choice is a surgical removal of the entire tumor for large-sized tumors. The prognosis of Superficial Angiomyxoma of Vulva is excellent with appropriate treatment, since it is a benign tumor

Who gets Superficial Angiomyxoma of Vulva? (Age and Sex Distribution)

• Superficial Angiomyxoma of Vulva is generally present in young and slightly middle-aged women. Women in the age category of 30-40 years are affected the most
• In general, superficial angiomyxomas may be found in a wider age range of individuals
• There is no geographical, racial, or ethnic preference noticed

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

• No specific risk factors are evident or identified for Superficial Angiomyxoma of Vulva
• However, some cases of cutaneous superficial angiomyxoma are seen to occur against a background of Carney complex (a genetic disorder)

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 Superficial Angiomyxoma of Vulva? (Etiology)

• The exact cause of Superficial Angiomyxoma of Vulva development is unknown
• Since the tumor responds to hormone therapy, the cause may be linked to an abnormal hormonal influence
• In general, superficial angiomyxoma of skin (which is the more common type of angiomyxoma), is associated with Carney complex (an autosomal dominant genetic disorder)

What are the Signs and Symptoms of Superficial Angiomyxoma of Vulva?

The signs and symptoms of Superficial Angiomyxoma of Vulva include:

• Superficial angiomyxomas are slow-growing masses that involve the skin and subcutaneous tissues below the skin
• The tumor size is mostly below 5 cm, though some may grow to larger sizes
• Most of the tumors appear as well-defined, painless masses shaped like a polyp
• There may be pain during sexual intercourse
• Large-sized tumors may cause pain and compress surrounding structures and organs
• Large tumors may also cause significant signs and symptoms such as abdominal/pelvic pain and discomfort, urination difficulties, and even lower back pain

How is Superficial Angiomyxoma of Vulva Diagnosed?

Superficial Angiomyxoma of Vulva diagnosis may involve the following tools:

• 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 Superficial Angiomyxoma of Vulva?

The possible complications of Superficial Angiomyxoma of Vulva may include:

• Emotional stress and concern, especially associated with tumors in the genital region
• Superficial vulvar tumors may present cosmetic concerns
• Tumor recurrence following surgery is known to take place in about 1 in 3 cases (due to their incomplete removal)
• Damage of vital nerves, blood vessels, and surrounding structures, during surgery to remove the tumors
• Post-surgical infection at the wound site is a potential complication

Vulvar Superficial Angiomyxoma is not an aggressive tumor and it does not destroy the adjacent tissue structures, unlike its aggressive counterpart.

How is Superficial Angiomyxoma of Vulva Treated?

Following are the treatment methods adopted for Superficial Angiomyxoma of Vulva:

• The healthcare provider may recommend a ‘wait and watch’ approach for small-sized tumors presenting mild signs and symptoms, after a diagnosis of superficial angiomyxoma has been established
• Hormonal therapy may be considered to slow the growth rate of tumor, to decrease the size of the tumor, and also to decrease the chances of recurrence or the tumor
• Surgical intervention with complete excision can result in a complete cure, and also eliminate the chances of tumor recurrence
• 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, since the tumor can recur in some cases

How can Superficial Angiomyxoma of Vulva be Prevented?

• Current medical research has not established a way of preventing Vulvar Superficial Angiomyxoma
• Medical screening at regular intervals with scans and physical examinations are advised

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

• The prognosis of Superficial Angiomyxoma of Vulva is generally excellent on surgical excision and removal of the tumor, since it is a benign tumor
• Periodic follow-up check-ups with screening are advised

Additional and Relevant Useful Information for Superficial Angiomyxoma of Vulva:

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

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