Cutaneous Superficial Angiomyxoma

Cutaneous Superficial Angiomyxoma

Article
Skin Care
Diseases & Conditions
+2
Contributed byLester Fahrner, MD+1 moreFeb 14, 2021

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

  • Cutaneous Myxoma
  • Dermal Superficial Angiomyxoma
  • Superficial Angiomyxoma of Skin

What is Cutaneous Superficial Angiomyxoma? (Definition/Background Information)

  • Cutaneous Superficial Angiomyxoma is an uncommon, benign, mucin-producing tumor of myxoid cells (hence termed ‘myxoma’), affecting the skin. It is also known as Superficial Angiomyxoma of Skin and is generally seen in middle-aged and older adults
  • It leads to the formation of flesh-colored nodules that may occur on any part of the body, such as on the head and neck region or arms and legs
  • The cause of Cutaneous Superficial Angiomyxoma is largely unknown, but it could be due to genetic mutations. A majority of the tumors occur sporadically and there are no well-established risk factors
  • In some cases, the tumor occurs in the presence of Carney complex (an inherited genetic disorder). The presence of superficial angiomyxomas may be a first sign of Carney complex in some individuals
  • Generally, small-size tumors that are asymptomatic may require no treatment, apart from close observation. Tumors that cause significant signs and symptoms may be surgically removed
  • The prognosis of Cutaneous Superficial Angiomyxoma is usually excellent with adequate treatment. Nevertheless, the overall outcome may also depend upon the successful treatment of the underlying Carney complex (if present)

Who gets Cutaneous Superficial Angiomyxoma? (Age and Sex Distribution)

  • Cutaneous Superficial Angiomyxoma is present during adulthood; an age range of 40-60 years is observed
  • Both males and females are affected and no preference is seen, though some studies report a male predominance
  • Individuals of all racial and ethnic background may be affected. Worldwide, no geographical localization of Superficial Angiomyxoma of Skin has been reported

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

  • Most cases of Cutaneous Superficial Angiomyxoma are known to occur in a sporadic manner. The risk factors for sporadic tumors are currently unidentified
  • Carney complex is a risk factor for Cutaneous Superficial Angiomyxoma in some of the cases. Carney complex is an inherited 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 one's 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 Cutaneous Superficial Angiomyxoma? (Etiology)

  • The cause of development of Cutaneous Superficial Angiomyxoma is unknown in a majority of cases. This soft tissue tumor may form and develop due to genetic mutations
  • Research is currently being undertaken to identify the relevant causal factors
  • The presence of multiple tumors is observed to be linked to Carney complex; a complex genetic disorder causing the formation of tumors in the body. Carney complex s inherited in an autosomal dominant manner

What are the Signs and Symptoms of Cutaneous Superficial Angiomyxoma?

The signs and symptoms of Cutaneous Superficial Angiomyxoma may include:

  • The presence of solitary or multiple cutaneous nodular lesions
  • 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; the common location of the tumor is usually the trunk
  • The tumor is usually painless and non-itchy; however, pain may be felt with pressure

When Superficial Angiomyxoma of Skin occurs along with Carney complex, then presentations of the underlying disorder may be noted. Also, studies inform that the common sites for tumor occurrence against a background of Carney complex are the outer ears and eyelids.

How is Cutaneous Superficial Angiomyxoma Diagnosed?

A diagnosis of Cutaneous Superficial Angiomyxoma may involve the following:

  • A thorough medical history and physical examination
  • 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
  • Genetic mutational analysis to determine the presence of Carney complex
  • Skin biopsy: A skin 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 Cutaneous Superficial Angiomyxoma?

Cutaneous Superficial Angiomyxoma is a rare skin lesion 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
  • Tumor recurrence following surgery is known to take place (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
  • Complications that arise due to underlying Carney complex

How is Cutaneous Superficial Angiomyxoma Treated?

The treatment of Cutaneous Superficial Angiomyxoma may involve the following:

  • In a majority of cases, removal of the tumor may not be necessary, unless it causes bothersome signs and symptoms such as cosmetic issues
  • 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
  • The treatment is a complete surgical excision, which results in a cure
  • 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 Cutaneous Superficial Angiomyxoma be Prevented?

Presently, the sporadic cases of Cutaneous Superficial 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

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

  • The prognosis for sporadic Cutaneous Superficial Angiomyxoma is excellent with appropriate treatment, since it is a benign tumor
  • The prognosis of angiomyxomas occurring against a backdrop of Carney complex may depend upon the severity of the underlying condition

Additional and Relevant Useful Information for Cutaneous Superficial Angiomyxoma:

  • There is no evidence to prove that oily foods and chocolate-based products have an influence on Superficial Angiomyxoma of Skin formation
  • Cleaning the skin too hard with strong chemicals or soaps may aggravate the skin condition. Care must be taken avoid strong soaps and chemicals that could potentially worsen the condition
  • The presence of dirt on the body is not a causative factor for the condition. However, it helps to be clean and hygienic, which may help the condition from getting worse
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On the Article

Krish Tangella MD, MBA picture
Approved by

Krish Tangella MD, MBA

Pathology, Medical Editorial Board, DoveMed Team
Lester Fahrner, MD picture
Reviewed by

Lester Fahrner, MD

Chief Medical Officer, DoveMed Team

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