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

• AFX (Atypical Fibroxanthoma)

What is Atypical Fibroxanthoma? (Definition/Background Information)

• Atypical Fibroxanthoma (AFX) is a benign, skin-based tumor mostly observed in elderly adults. The tumor develops on sun-exposed skin surfaces and is generally caused by ultraviolet radiation. Even though AFX may raise a suspicion for a malignancy, it is typically benign
• Atypical Fibroxanthoma tumors appear as well-defined and small nodules, which are mostly painless. They form on the head and neck area, in most cases, and may ulcerate and bleed
• Complete excision by surgery is curative and the prognosis of Atypical Fibroxanthoma is usually excellent. However, occasionally the tumors are known to recur

Who gets Atypical Fibroxanthoma? (Age and Sex Distribution)

• Atypical Fibroxanthoma is observed in a higher incidence in elderly men and women in the 60-80 year’ age range. But, it may be observed in individuals of a wide age category (13-95 years reported)
• A male predilection is noted, even though both male and female genders are affected
• Almost all cases are observed in white-skinned individuals. Generally, the tumor is not found outside the Caucasian race

What are the Risk Factors for Atypical Fibroxanthoma? (Predisposing Factors)

The risk factors for Atypical Fibroxanthoma include:

• Severe sun-exposure; exposure to sun’s ultraviolet radiation
• Exposure to other forms of radiation, such as for therapeutic reasons (like radiation therapy)
• Individuals living in tropical regions, where direct sun-exposure is high
• Fair-skinned Caucasians have a much higher risk incidence than dark-skinned individuals

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 Atypical Fibroxanthoma? (Etiology)

• Atypical Fibroxanthoma forms on skin that is significantly damaged from chronic exposure to sunlight
• It can also develop due to longstanding radiation therapy, or due to a combination of the two factors
• The damage to the skin induces abnormal formation of spindle (tumor) cells, within the skin layers

What are the Signs and Symptoms of Atypical Fibroxanthoma?

The signs and symptoms of Atypical Fibroxanthoma may include:

• The head and neck area is usually affected in 80% of the cases. The tumor may particularly form on the back of the neck, nose, ears, or on the cheeks
• The presence of a single or multiple tumor nodules may be seen; the tumors are clearly-defined
• The growth of the nodules is quite rapid following onset
• The size of the skin lesion is between 2-3 cm; tumors over 3 cm are uncommon
• The raised skin lesion is usually deep red in color. The skin around the nodule may present sun-damaged appearance
• The lesions may ooze fluid and bleed
• Often, ulceration is noted, following which curst formation takes place

How is Atypical Fibroxanthoma Diagnosed?

A diagnosis of Atypical Fibroxanthoma is made using the following tools:

• Physical examination with evaluation of the individual’s medical history
• 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
• 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

A differential diagnosis to eliminate the following tumors may be undertaken prior to arriving at a definite diagnosis:

• Leiomyosarcoma of skin
• Poorly-differentiated squamous cell carcinoma
• Spindle cell melanoma
• Superficial form of malignant fibrous histiocytoma

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 Atypical Fibroxanthoma?

The complications from Atypical Fibroxanthoma could include:

• AFX can give rise to cosmetic concerns and emotional stress
• Initially, the lesion may rise a concern for malignancy
• Local recurrence after surgery; however, this is reported to be a very rare event

How is Atypical Fibroxanthoma Treated?

Treatment measures for Atypical Fibroxanthoma include the following:

• Surgical excision and removal of the tumor is generally considered by the healthcare provider
• Mohs micrographic surgery: It is an alternative technique to standard excision for tumors that are large or recurrent
  • A thin layer of skin is removed and checked for cancer
  • This process is then carried out continuously, until a tumor-free slice of skin is obtained
  • This procedure can help conserve normal healthy skin and soft tissue
• Smaller lesions are treated using a procedure called curettage, wherein a special instrument is used to scoop-out the tumor
• 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 Atypical Fibroxanthoma be Prevented?

A few methods to prevent Atypical Fibroxanthoma include:

• Avoid prolonged and chronic exposure to the sun
• Take adequate safety steps to reduce exposure to the UV rays, by using sunscreens with high sun-protection factor, wide-brimmed hats, and protective clothing
• During summer, adequate sun-exposure and oral steroids, a few days before vacationing in the tropical areas can help prevent the development of AFX
• Avoid excessive sunbathing (particularly if you are fair-skinned), use of tanning beds, sun lamps, and chemical agents, that accelerate sun tanning
• Individuals, who are regularly exposed to the sun or work under the sun, should get their skin periodically examined by a physician. This is crucial if they suspect (or if there are) any noticeable skin changes
• Keep yourself well-hydrated, if sun-exposure for prolonged periods is unavoidable

What is the Prognosis of Atypical Fibroxanthoma? (Outcomes/Resolutions)

The prognosis of Atypical Fibroxanthoma is excellent with appropriate surgical intervention, since it is a benign condition. AFX recurrence risk is normally low.

Additional and Relevant Useful Information for Atypical Fibroxanthoma:

Some associate the term superficial malignant fibrous histiocytoma (MFH) with the tumor. However, this is incorrect, since Atypical Fibroxanthoma is not so very aggressive.