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

• Angiomyxoma, NOS

What is Angiomyxoma? (Definition/Background Information)

• An Angiomyxoma is an uncommon, benign, mucin-producing soft tissue tumor of myxoid cells (hence termed ‘myxoma’). It is classified into 2 main types:
  • Superficial Angiomyxoma: It is generally observed on the skin or just below the skin (superficial location), anywhere in the body. The tumor is slow-growing, docile, and affects middle-aged and older adults
  • Aggressive Angiomyxoma (AA): It is a slow-growing tumor having no metastatic potential, but is found to be locally aggressive with high-infiltrative capacity (spreading to adjacent tissues and structures). Women in the child-bearing phase are most prone to this tumor type
• The cause of Angiomyxoma is largely unknown, but it could be due to genetic mutations. In some cases, superficial angiomyxomas occur in the presence of Carney complex (a type of genetic disorder)
• Generally, small-sized Superficial Angiomyxomas that are asymptomatic may require no treatment, apart from close observation. Superficial Angiomyxomas that cause significant signs and symptoms, and a majority of the Aggressive Angiomyxoma are surgically treated
• The prognosis of Angiomyxoma is excellent with adequate treatment in a majority of cases. Nevertheless, the prognosis depends upon various factors including the type of the tumor, its location, the size of the tumor, and the overall health status of the individual. The prognosis of Superficial Angiomyxoma is better than that of Aggressive Angiomyxoma

Who gets Angiomyxoma? (Age and Sex Distribution)

• Angiomyxomas are seen in the adult population and both males and females are affected
  • Cutaneous Superficial Angiomyxoma is present during adulthood; an age range of 40-60 years is observed
  • Deep Aggressive Angiomyxoma mostly affects women, between the ages of 20-50 years. Females are affected much more than males in a 6:1 ratio
• Individuals of all racial and ethnic background may be affected. Worldwide, no geographical localization of Angiomyxoma has been reported

What are the Risk Factors for Angiomyxoma? (Predisposing Factors)

The risk factors for Angiomyxoma may include the following factors:

• Carney complex is a risk factor for Cutaneous Superficial Angiomyxoma in some of the cases. Carney complex is an inherited genetic disorder
• Women in the child-bearing age group are very vulnerable to Aggressive Angiomyxoma

Nevertheless, currently, no risk factors have been noted for sporadic cases of both superficial and aggressive Angiomyxomas.

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 Angiomyxoma? (Etiology)

• The cause of development of Angiomyxoma is unknown. This soft tissue tumor may form and develop due to genetic mutations
• Research is currently being undertaken to identify the relevant causal factors

What are the Signs and Symptoms of Angiomyxoma?

In general, the signs and symptoms of Angiomyxoma may include the following:

• The presence of solitary or multiple subcutaneous nodular lesions
• The tumors are slow-growing and may be asymptomatic during the initial stages
• These soft tissue tumors are non-itchy, but may present with pain and tenderness upon touch

The signs and symptoms of Cutaneous Superficial Angiomyxoma may additionally include:

• The nodules are flesh-colored and measure in size from 0.5 to 3 cm
• The tumor may form in the head and neck area, chest or back, and arms and legs
• When Superficial Angiomyxoma of Skin occur along with Carney complex, then presentations of the underlying disorder may be noted

The signs and symptoms of Aggressive Angiomyxoma may additionally include:

• They may remain undetected during the initial formative phase, until they reach about 10 cm in size
• AA mainly occurs in the pelvic or groin area (perineal region). It has also been observed within the abdominal cavity
• The presence of the tumor can lead to a painful sexual intercourse

How is Angiomyxoma Diagnosed?

The diagnosis of Angiomyxoma depends upon the location and type of the tumor. Superficial Angiomyxomas are diagnosed through skin exam tools and skin biopsy, while Aggressive Angiomyxomas are diagnosed through a thorough physical (pelvic) exam, radiological imaging studies, and a tissue biopsy.

The diagnosis of Angiomyxoma may involve the following tests and exams:

• A thorough medical history and physical examination; a pelvic exam may be undertaken for Aggressive Angiomyxoma, since the pelvis/perineum is the most common location for this tumor type
• Dermoscopy: It is a diagnostic tool where a dermatologist examines the skin using a special magnified lens
• Wood’s lamp examination: In this procedure, the healthcare provider examines the skin using ultraviolet light. It is performed to examine the change in skin pigmentation
• Radiological imaging studies of the affected region, such as through CT or MRI scans
• Genetic mutational analysis to determine the presence of Carney complex
• Tissue biopsy: A tissue biopsy is performed and sent to a laboratory for a pathological examination. The 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

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 Angiomyxoma?

Angiomyxoma is an uncommon benign tumor that may cause the following complications:

• Emotional stress and concern associated with cancer
• The tumors can ulcerate and bleed. This can cause secondary bacterial or fungal infections to develop (superimposed infections)
• Some individuals may have concerns regarding their cosmetic appearance, especially when the tumors occur in the head and neck area, particularly in the case of Superficial Angiomyxomas
• Damage of vital nerves, blood vessels, and surrounding structures, during surgery to remove the tumors: Mainly in the case of Aggressive Angiomyxoma, if the tumor is large and has infiltrated deep into adjoining regions, it may lead to significant risks during surgical operations
• Post-surgical infection at the wound site is a potential complication
• Tumor recurrence following surgery is known to take place (due to their incomplete removal), both in the case of superficial and aggressive Angiomyxomas
• Complications that arises due to underlying Carney complex

How is Angiomyxoma Treated?

The treatment of Angiomyxoma may involve the following:

• In a majority of cases, removal of Cutaneous Superficial Angiomyxoma may not be necessary, unless it causes bothersome signs and symptoms such as cosmetic issues
• If there is any pain, it is controlled through pain medications
• Hormonal therapy may be considered (in women) 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
• Surgery:
  • For Cutaneous Superficial Angiomyxoma presenting significant symptoms or cosmetic concerns, the treatment is a complete surgical excision, which can result in a cure
  • For Aggressive Angiomyxoma, wide surgical excision with removal of the entire lesion is the standard treatment mode. If the tumor is not fully removed, then there is a high chance of recurrence
• Treatment of underlying Carney complex may have to be undertaken (if present)
• Follow-up care with regular screening and check-ups are important and encouraged

How can Angiomyxoma be Prevented?

Presently, the sporadic cases of Angiomyxoma cannot be prevented. Also, there are no specific methods or guidelines to prevent tumor formation, if it occurs in association with Carney complex, which is a genetic condition.

• Genetic testing of the expecting parents (and related family members) and prenatal diagnosis (molecular testing of the fetus during pregnancy) may help in understanding the risks better during pregnancy
• If there is a family history of the condition, then genetic counseling will help assess risks, before planning for a child
• Active research is currently being performed to explore the possibilities for treatment and prevention of inherited and acquired genetic disorders such as Carney complex
• Regular medical screening at periodic intervals with blood tests, radiological scans, and physical examinations, are mandatory for those who have been diagnosed with the tumor
• Due to chances of its recurrence, often several years of active follow-up vigilance is necessary

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

The prognosis of Angiomyxoma depends upon the type (whether superficial or aggressive), the size and location of the tumor, and overall health of the individual. But, in general, the prognosis is excellent in a majority of cases, since Angiomyxomas are benign tumors.

• The prognosis for sporadic Cutaneous Superficial Angiomyxoma is excellent with appropriate treatment, since it is a benign tumor
• The prognosis of Cutaneous Superficial Angiomyxoma occurring against a backdrop of Carney complex may depend upon the severity of the underlying condition
• The prognosis of Aggressive Angiomyxoma is excellent when lesions are small and located in a surgically accessible area, without much local infiltration. For tumors that are diagnosed in their advanced stages, surgical excision is difficult and intricate, especially if the tumor has generally spread around the region

Additional and Relevant Useful Information for Angiomyxoma:

There is no evidence to prove that oily foods and chocolate-based products have an influence on Angiomyxoma formation.