Lymphangioma is developed from original lymphatic sac and vessel in mesenchymal tissue. It is tumor-like malformation initiated after the original sac in some parts is isolated from lymphatic system during embryonic development. As a congenital benign hamartoma, it is divided into three subtypes: simple lymphangioma, cavernous lymphangioma, and cystic lymphangioma. 75% of these lesions occur in neck, 20% in auxiliary region, and only 5% in abdominal cavity. Retroperitoneal lymphangioma accounts for less than 1% of all lymphangiomas (Bhavsar et al. 2010). More than 90% of cases occur in people aged ≤2 years. The prevalence is similar between men and women. Localized lymphangioma has a good prognosis after surgery, with a low relapse rate. Diffuse lymphangioma is usually ill-defined, cannot be completely removed, and relapses repeatedly. Elephantiasis of limbs poorly responds to the treatment. Notably, lymphangioma will not become malignant.
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