Adult Primary Retroperitoneal Lymphangioma: Updated Facts

Abstract

Lymphangioma is a rare, benign tumor of the lymphatic system. It is believed to be a congenital malformation, when part of the lymphatic channels fail to connect to the main lymphatic system. Lymphangioma is a tumor of the pediatric age, with 50% of patients presenting at birth. The head and neck are the main affected sites (75%), while the retroperitoneal cavity is the least affected area, and comprises less than 1% of cases. Adult lymphangioma is an extremely rare tumor, and adult retroperitoneal lymphangioma (ARL) is even a rarer tumor. Over the last two decades, we have experienced a significant increase in reports published in the English literature discussing ARL. As reports have increased, several questions about previously known facts regarding this tumor arose: For years, it was known that ARL is usually an asymptomatic tumor which is incidentally found - is it a true claim? Is abdominal magnetic resonance imaging the radiological test of choice for diagnosis? What is the best therapeutic option? The main aim for this article is to review the current and old English literature concerning ARL, in order to collect data regarding demographic features, clinical presentation, imaging tests used for diagnosis, therapeutic options and follow-up. This in turn will give precise updated answers for the previous questions. In addition, it will raise awareness for the treating physician regarding the most effective approach for early diagnosis and best therapeutic option to be selected.