Diffuse Infantile Fibromatosis

Diffuse Infantile Fibromatosis is a rare and benign, yet aggressive, fibrous tumor that predominantly affects infants and young children. This condition is characterized by the proliferation of fibroblasts, which are cells that produce collagen and other fibers, leading to the development of firm and diffuse masses within the affected tissues. The etiology of diffuse infantile fibromatosis remains largely unknown, although genetic and environmental factors may play a role in its development.

Symptoms and Diagnosis

The clinical presentation of diffuse infantile fibromatosis can vary significantly depending on the location and size of the tumor. Common symptoms include a palpable, non-tender mass that may be associated with overlying skin changes. These tumors most frequently occur in the deep soft tissues, including the muscles, tendons, and aponeuroses, but can also affect the visceral organs.

Diagnosis of diffuse infantile fibromatosis is primarily based on histological examination of the tumor. Imaging techniques such as magnetic resonance imaging (MRI) and computed tomography (CT) scans can be useful in assessing the extent of the disease and planning surgical intervention. Biopsy and histopathological analysis are crucial for confirming the diagnosis, with typical findings including the proliferation of spindle-shaped fibroblasts and a collagenous stroma.

Treatment and Prognosis

The mainstay of treatment for diffuse infantile fibromatosis is surgical resection with the aim of complete removal of the tumor. However, due to the infiltrative nature of the disease, achieving clear margins can be challenging, and recurrence is common. In cases where surgical resection is not feasible or in instances of recurrence, other treatment modalities such as chemotherapy and radiation therapy may be considered, although their efficacy is variable.

The prognosis for patients with diffuse infantile fibromatosis is generally favorable, as the condition is benign and does not metastasize. However, the aggressive growth of the tumor can lead to significant morbidity, particularly if critical structures are involved or if complete resection is not possible.

Epidemiology

Diffuse infantile fibromatosis is a rare condition, with a limited number of cases reported in the medical literature. It can occur in both genders and across various ethnic groups, although some studies suggest a slight predilection for females. The majority of cases are diagnosed in infancy or early childhood.

See Also

• Fibromatosis
• Fibrous tumor
• Pediatric oncology

References

• Need help finding a doctor or specialist anywhere in the world? WikiMD's DocFinder can help with millions of doctors!

This article is a stub. Help WikiMD grow by registering to expand it. Editing is available only to registered and verified users. About WikiMD: A comprehensive, free health & wellness encyclopedia.