Aggressive infantile fibromatosis

Aggressive infantile fibromatosis (AIF) is a rare, non-malignant tumor that primarily affects children. Despite its benign nature, the tumor can be invasive and aggressive, often leading to significant morbidity. It is a subtype of fibromatosis, a group of conditions characterized by the growth of fibrous tissue.

Etiology[edit | edit source]

The exact cause of aggressive infantile fibromatosis is unknown. However, it is believed to be associated with certain genetic mutations. Some studies suggest a link with mutations in the CTNNB1 gene, which is involved in cell adhesion and signal transduction.

Clinical Presentation[edit | edit source]

Patients with aggressive infantile fibromatosis typically present with a painless, firm mass. The tumor can occur anywhere in the body but is most commonly found in the head and neck region, followed by the trunk and extremities. Despite being non-malignant, the tumor can invade surrounding tissues and cause functional impairment.

Diagnosis[edit | edit source]

Diagnosis of aggressive infantile fibromatosis is primarily based on histopathological examination. Imaging studies such as MRI and CT scan can be used to assess the extent of the tumor. Biopsy of the tumor is necessary for definitive diagnosis.

Treatment[edit | edit source]

Treatment of aggressive infantile fibromatosis is challenging due to its aggressive nature and tendency to recur. Surgical resection is the primary treatment modality. However, complete resection is often difficult due to the invasive nature of the tumor. Other treatment options include chemotherapy, radiotherapy, and targeted therapies.

Prognosis[edit | edit source]

The prognosis of aggressive infantile fibromatosis is variable. While the tumor is non-malignant and does not metastasize, it can cause significant morbidity due to local invasion and recurrence. Long-term follow-up is necessary to monitor for recurrence.