Benign lipoblastomatosis

Benign Lipoblastomatosis

Benign lipoblastomatosis is a rare, non-cancerous tumor that primarily affects infants and young children. It is characterized by the proliferation of immature fat cells, known as lipoblasts, within the soft tissues. This condition is considered a type of lipoma, but it is distinct due to its unique histological features and clinical behavior.

Clinical Presentation

Benign lipoblastomatosis typically presents as a painless, soft, and slowly enlarging mass. It most commonly occurs in the extremities, trunk, or neck of children under the age of three. The tumor is usually well-circumscribed but can infiltrate surrounding tissues, making complete surgical excision challenging.

Pathophysiology

The exact cause of benign lipoblastomatosis is not well understood. It is believed to arise from a developmental anomaly in adipose tissue. Histologically, the tumor is composed of a mixture of mature adipocytes and immature lipoblasts, with a myxoid stroma. The presence of lipoblasts is a key feature that differentiates it from other types of lipomas.

Diagnosis

Diagnosis of benign lipoblastomatosis is primarily based on histological examination of the excised tumor. Imaging studies such as MRI or CT scan may be used to assess the extent of the tumor and its relationship to surrounding structures. A biopsy may be performed to obtain a tissue sample for pathological analysis.

Treatment

The mainstay of treatment for benign lipoblastomatosis is surgical excision. Due to the infiltrative nature of the tumor, achieving clear margins can be difficult, and there is a risk of recurrence. In some cases, multiple surgeries may be required. Regular follow-up is essential to monitor for recurrence.

Prognosis

The prognosis for benign lipoblastomatosis is generally favorable, as it is a benign condition. However, the potential for recurrence necessitates careful long-term follow-up. Malignant transformation is extremely rare.

Also see

• Lipoma
• Adipose tissue
• Pediatric tumors
• Soft tissue tumors

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Source: Data courtesy of the U.S. National Library of Medicine. Since the data might have changed, please query MeSH on Benign lipoblastomatosis for any updates.

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