Acral Fibromyxoma

Acral Fibromyxoma

Article
Bone, Muscle, & Joint
Diseases & Conditions
+2
Contributed byKrish Tangella MD, MBAMar 25, 2018

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

  • AFM (Acral Fibromyxoma)
  • Digital Fibromyxoma
  • Superficial Acral Fibromyxoma (SAFM)

What is Acral Fibromyxoma? (Definition/Background Information)

  • Acral Fibromyxoma (AFM) is an uncommon, benign tumor of the soft tissues, mostly present around or below the fingernail or toenail. The cause of tumor formation is unknown and no particular risk factors have been identified
  • Middle-aged and elderly individuals are prone to Acral Fibromyxomas. The tumors may be painful or painless, appearing as a single small lump in the hand or feet
  • The treatment may involve a complete surgical removal of the tumor, in case of significant signs and symptoms. This can ensure a cure
  • With adequate treatment, the prognosis for Acral Fibromyxoma is generally excellent, since it is a benign tumor. The risk of recurrence of the tumor is low

Who gets Acral Fibromyxoma? (Age and Sex Distribution)

  • Acral Fibromyxoma is observed in a wide age range - between 4 and 85 years. However, a majority of the tumors (nearly 70% of the cases) are observed are individuals in their mid-adult to late-adult phase (40 years and older)
  • Both males and females are affected, though there is a slight male predominance (2:1 male-female ratio)
  • There is no known ethnic or racial preference
  • Acral Fibromyxomas are very rare tumors; only about 50-60 cases have been reported in the medical records

What are the Risk Factors for Acral Fibromyxoma? (Predisposing Factors)

  • The specific risk factors for Acral Fibromyxoma have not been identified
  • Some cases have been recorded against a background of trauma; but, the relation has not been clearly established

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 Acral Fibromyxoma? (Etiology)

  • The exact cause and mechanism of formation of Acral Fibromyxoma is unknown
  • Currently, research is being undertaken to understand how these tumors form and develop

What are the Signs and Symptoms of Acral Fibromyxoma?

The signs and symptoms of Acral Fibromyxoma may include the following:

  • Almost all tumors are located on the hands or feet: The fingers and toes are involved in over 65% of the cases, while other sites include the palms of the hand and soles of the feet
  • On the digits of the hand or feet, the portion around/below the nail is involved (the fingernail and toenail is involved). Most Acral Fibromyxomas are observed on the large toe
  • Presence of a dome-shaped mass that is moderately-circumscribed with ill-defined edges
  • Single, slow-growing masses that are observed superficially (on or just below the skin surface). Some tumors may grow over a period of several decades (even up to 30 years)
  • The tumors may cause pain, which is observed in about 40% of the cases
  • Periungual or subungual tumors may cause deformities of the nail that is noted in many cases
  • Ulceration of the overlying skin may be seen; larger tumors may invade into the deeper subcutaneous tissues and impinge on the bone
  • In some cases, indentations may be observed on the underlying bony surface

Small-sized tumors may have mild signs and symptoms, while some may be asymptomatic during the initial period.

How is Acral Fibromyxoma Diagnosed?

A diagnosis of Acral Fibromyxoma may involve:

  • Complete physical examination and evaluation of the individual’s medical history
  • X-ray of the hand or foot that is involved, to study the extent of bone involvement
  • CT scan or MRI scan of the affected region, if necessary
  • Tissue biopsy: A tissue biopsy is performed and sent to a laboratory for a pathological examination, who 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 Acral Fibromyxoma?

The complications due to Acral Fibromyxoma may include:

  • Secondary infections due to skin ulceration
  • Discomfort and pain may affect one’s daily routine and affect the quality of life
  • Recurrence of Acral Fibromyxoma after surgery can take place, if the tumor is incompletely excised; about 1 in 5-6 cases are known to recur

How is Acral Fibromyxoma Treated?

The treatment of Acral Fibromyxoma may be undertaken as follows:

  • If there are no symptoms associated with AFM, then non-surgical treatment measures may be adopted including pain management
  • A complete surgical removal of the lesion can result in a cure. On a complete surgical excision with clear margins, the tumor is generally not known to recur
  • Follow-up care with regular screening and check-ups are important

How can Acral Fibromyxoma be Prevented?

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

What is the Prognosis of Acral Fibromyxoma? (Outcomes/Resolutions)

  • The prognosis for Acral Fibromyxoma is excellent with surgical intervention, since these are benign tumors. With complete excision and removal, the recurrence risk is minimal to absent
  • Asymptomatic and benign lesions may require no treatment, unless they affect the quality of life
  • Acral Fibromyxomas are not known to undergo malignant transformations, and to date, no metastasis has been reported in the medical literature

Additional and Relevant Useful Information for Acral Fibromyxoma:

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

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

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Krish Tangella MD, MBA picture
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Krish Tangella MD, MBA

Pathology, Medical Editorial Board, DoveMed Team

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