Retroperitoneal lymphangioleiomyomatosis: CT appearance.

Lymphangioleiomyomatosis is a rare disease involving the lung, lymphatic trunk and lymph nodes. Sometimes it appears in the retroperitoneum with or without subsequent development in the lungs. We present a 52-year-old woman with retroperitoneal location of the disease. We discuss the abdominal CT appearance, the cytologic and immunophenotypic features and a preoperative differential diagnosis is attempted.