Lipofibromatosis

Lipofibromatosis is a rare, benign tumor that primarily affects children and young adults. It is characterized by the proliferation of mature adipose and fibrous tissue.

Epidemiology

Lipofibromatosis is a rare condition, with fewer than 50 cases reported in the medical literature. It affects both males and females equally and can occur at any age, but it is most commonly diagnosed in children and young adults.

Pathology

Lipofibromatosis is characterized by the proliferation of mature adipose and fibrous tissue. The tumor is typically well-circumscribed and can vary in size. Histologically, it is composed of mature adipocytes and fibroblasts, with a variable amount of collagen. The tumor cells do not show significant atypia or mitotic activity, which helps distinguish lipofibromatosis from malignant liposarcoma.

Clinical Presentation

Patients with lipofibromatosis typically present with a painless, slow-growing mass. The tumor can occur anywhere in the body, but it is most commonly found in the extremities, particularly the hands and feet. Other less common sites include the trunk, head and neck, and internal organs.

Diagnosis

The diagnosis of lipofibromatosis is typically made based on the clinical presentation and histological findings. Imaging studies, such as magnetic resonance imaging (MRI), can be helpful in determining the extent of the tumor and planning for surgical resection. A biopsy of the tumor is usually performed to confirm the diagnosis.

Treatment

The primary treatment for lipofibromatosis is surgical resection of the tumor. Complete resection is often curative, but the tumor can recur if not completely removed. In cases where complete resection is not possible, close follow-up is recommended to monitor for tumor growth.

Prognosis

The prognosis for patients with lipofibromatosis is generally good, as the tumor is benign and does not metastasize. However, the tumor can cause significant morbidity if it involves critical structures or if it recurs after incomplete resection.