Angiomatosis of soft tissue

Angiomatosis of soft tissue (AST) is a rare, benign vascular anomaly primarily characterized by extensive mesenchymal hyperplasia with a predilection for the lower extremities. It often presents in early life, complicating the diagnosis because of overlapping clinical and radiological features with other vascular malformations. The etiology of AST remains largely unknown, although somatic mutations, particularly in the PIK3CA gene, have been identified. Clinically, patients typically exhibit persistent swelling and pain, with imaging modalities revealing infiltrative soft tissue masses. Diagnosis is frequently confirmed postoperatively as specific criteria remain absent. Differential diagnoses include conditions such as venous malformations and fibro-adipose vascular anomalies. Current treatment mainly involves surgical excision; however, the high recurrence rates necessitate further exploration of targeted therapies. This review aimed to systematically discuss the current understanding of AST, emphasizing the need for improved diagnostic and management strategies.