Retroperitoneal Cystic Lymphangioma: A Case Report of Spontaneous Regression and Review of Literature

Abstract

Background: Cystic lymphangioma (CL) is a rare and benign tumor derived from the lymphatic system that can have different localisations. This tumor consists of several numbers of cysts that have cavity-like appearance. During childhood, cystic lymphangiomas are as common in boys as in girls (sex ratio 1:1). This lesion can have a sudden onset, a rapid growth or stability and can even regress spontaneously. It has a variable clinical presentation and sometimes can be asymptomatic. For this rare malformation the final diagnosis is a histological confirmation. Case presentation: We present the case of a 7-year-old girl that was diagnosed before birth with a retroperitoneal CL by ultrasound and Magnetic Resonance Imaging (MRI) with a left lumbar subcutaneous extension associated with a cutaneous lymphangioma. This retroperitoneal lesion remained asymptomatic, non visible clinically and it had a spontaneous resolution without any treatment; however, the cutaneous lesion kept evol-ving. Conclusion: The diagnosis of CL can be made by imaging during the prenatal period and sometimes during adulthood. The usual treatment for asymptomatic CL is regular clinical and radiological monitoring. However for symptomatic lesions the first line treatment is surgery. In our case, surgical treatment was performed only for the skin lesions.