Familial renal retroperitoneal lymphangiomatosis: personal experience and review of literature.

Lymphangiomatosis of the kidneys and perirenal-retroperitoneal tissues is a rare disease of unknown etiology. We present two cases affecting members of the same family, supporting the familial nature of the disease. The natural history and related urological and systematic complications of the disease during a long-term follow-up are highlighted, while a comprehensive literature review is presented.