Introduction
Infantile fibromatosis, often known as IDF, is a benign tumor of myofibroblasts (cells of smooth muscle) that can develop in infants on the tips of their fingers and toes. IDF is relatively uncommon. In 1965, Reye established the first classification for these tumors, recognizing that they may be differentiated from many other fibrous growths, so this tumor is also known to be Reye's tumor. The IDF, also known as multiple hyaline fibromatosis, infantile dermal fibromatosis, subdermal fibromatosis tumor of infancy, fibroma durum multiplex, recurrent digital fibroma, and juvenile dermatofibroma, is a rare tumor that often affects one or more digits.
What Is an Infantile Digital Fibroma?
Infantile digital fibroma is an uncommon benign tumor that typically develops within the first two years of life, but it may also be present in up to one-third of patients at birth. It is usually a single lesion, but it might be multiple. The nodules of fibroma are typically hard and usually less than 2 cm in diameter, often painless, although some cases present joint dysfunction or discomfort. Larger tumors may also begin to compromise adjacent digits, resulting in a long-term functional abnormality hampering regular activities. Infantile digital fibromatosis equally affects both sexes, male and female. Most nodules emerge within the first few months of birth; one-third are congenital (present at birth), and 75 to 80 percent are discovered within the first year. Rarely do cases of infantile digital fibromatosis manifest in older kids or adults.
How Common Is Infantile Digital Fibroma?
Infantile digital fibroma is a relatively uncommon condition. Approximately 200 instances have been reported throughout the world.
How Does an Infantile Digital Fibroma Appear?
Infantile digital fibromatosis appears as a single or cluster of pink, complex, or gelatinous nodules on a baby's fingers or toes. Infantile digital fibromatosis lesions can grow up to several centimeters. The affected digit frequently takes on a deformed appearance; however, these conditions rarely lead to a loss of functionality. Lesions are more likely to appear on the fingers than on the toes, but they primarily affect the thumbs or the great toes. It is unusual for there to be more than one digit involved. Hands, feet, arms, nose, breasts, torso, and tongue are some of the uncommon extradigital locations that have been reported.
What Are the Symptoms of Infantile Digital Fibroma?
The infantile digital fibroma is usually asymptomatic and does not have any pain except, in some cases, might experience discomfort due to the growth of the fibroma. In rare situations, symptoms include reduced or complete loss of joint function and pain. Additionally, tumors may start to affect nearby digits with long-term functional impairment. The fibroma may grow slowly over time or rapidly, although neither growth pattern has any clinical importance. Even though there is little risk of metastasis and the lesion is benign, it frequently recurs in most cases. Some cases of spontaneous regression have also been reported to occur when left untreated.
What Are the Causes of Infantile Digital Fibroma?
No one knows for sure what makes infantile digital fibromatosis happen. People used to think that actin filaments are not in the right place of arrangement in myofibroblasts (type of muscle cells), and also It has been thought that the presence of these lesions at the sites of digital septation could be due to a possible dysregulation of morphogenetic protein, which is a transforming growth factor β1.
How to Diagnose an Infantile Digital Fibroma?
A skin biopsy can provide diagnostic confirmation. The hallmark histology of infantile digital fibroma is interwoven spindle-shaped cells and collagen fibers in the dermis (middle layer of skin) and subcutaneous tissue (under the skin). The cells contain peculiar inclusion structures and are believed to be myofibroblasts (the cell type that makes muscle fibers).
What Is the Treatment for Digital Fibroma in Infants?
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Medical Care: If the fibromas are not creating any issues, it is better to take a cautious approach and check their progress over the next few years. Many fibromas eventually resorb and disappear on their own. They can be removed with a simple surgical procedure in which the lump is shaved off. However, even after surgery, many of them reappear.
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Surgery: It has been estimated that 60 to 90 percent of infantile digital fibromatosis lesions will recur after undergoing local excision; nonetheless, surgery is only suggested in cases with functional impairment. In a handful of the cases that called for surgery, Mohs micrographic surgery with smooth muscle actin or trichrome staining is believed to be successful.
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Cryotherapy: It has also proven to be effective in some cases.
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Medication: There is no evidence that topical corticosteroids, either with or without occlusion, are effective in treating infantile digital fibromatosis. On the other hand, intralesional corticosteroids or Fluorouracil proved effective in some cases by inhibiting cell growth and proliferation. However, as there is no information regarding the medication's systemic absorption after being injected into these tumors, Fluorouracil injections should only be used to treat tiny tumors localized in one area.
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Patient Care and Management: Patients are checked frequently to look for any changes in symptoms or regression. The potential for regression to take place over a number of years may vary. In the event of discomfort or a restriction in joint movement that becomes apparent, imaging examinations and consultation with a surgeon are advised.
What Will Be the Treatment Outcome of Infantile Digital Fibroma?
Infantile digital fibromatosis has a good treatment outcome except for recurrence after surgery in some cases. Infantile digital fibromatosis is benign; there is no sign of metastases or cancerous change. Lesions caused by infantile digital fibromatosis regress on their own without leaving scars. Rarely, the lesions might result in deformity or functional disability.
Conclusion
Infantile digital fibroma is a pediatric soft tissue fibromatosis that is completely benign but has a high recurrence rate. We suggest a conservative watch-and-wait policy for patients with histologically confirmed infantile digital fibroma nodules that do not cause joint deformity or functional impairment because of the benign nature of the lesions and the high rate of postsurgical recurrence. If the patient develops deformities linked with the lesions, one should be willing to reach out to the doctor as soon as possible and get help.
