Lymphangiomatosis is a rare and benign mesenchymal disorder where there is developmental malformation and dilatation of lymphatic channels. The most common sites for lymphangiomatosis are the cervical neck and axilla, but it can also occur in the mediastinum, retroperitoneum, mesentery, omentum, colon, and pelvis. Renal lymphangiomatosis is an even rarer multicystic disorder which may be hilar, pericalyceal, paracalyceal, peripelvic, or para pelvic.
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