Lymphangioleiomyomatosis - Retroperitoneum.

ABSTRACT

Lymphangioleiomyomatosis (LAM) is a progressive disease affecting women of childbearing age. It is microscopically characterized by abnormal smooth muscle proliferation in the walls of lymphatics and lymph nodes. A 52-year old female presented with pain in left leg and bilateral pedal oedema. USG and CT abdomen revealed a retroperitoneal cystic mass suspicious of malignancy. Retroperitoneal exploration was done and the mass was excised which revealed milky contents within. On gross examination of the specimen, a spongy mass was noted . Histopathological examination led to a diagnosis of lymphangioleiomyomatosis. LAM is a rare disease unfamiliar to many physicians and may pose a diagnostic dilemma to the physician, possibly resulting in delayed or missed diagnosis . Many treatment modalities including corticosteroids, cytotoxic drugs, chemotherapy, radiation and hormonal therapy are suggested. The prognosis of LAM varies with the individual, but many patients respond well to intramuscular medroxyprogesterone injections.