Lymphangioma in Patients with Pulmonary Lymphangioleiomyomatosis: Results of Sirolimus Treatment

Lymphangioleiomyomatosis (LAM) is a rare disease caused by abnormal proliferation of smooth muscle-like cells mainly in lungs and axial lymph nodes, that belongs to the group of PEComa tumours.

Chylous pleural and abdominal effusions, lymhangioleiomiomas, angiomyolipomas are noticed in disease course. Sirolimus has been approved for LAM treatment, and also it has been reported to decrease the size of angiomyolipomas, lymphangiooedma, pleural and peritoneal chylous effusion.

The aim of the study was to assess the significance of sirolimus therapy in patients with LAM and lymphangioma in retroperitoneal space. Method: Fourteen women with confirmed diagnosis of LAM (13 with sporadic LAM, and one TSC/LAM) and presence of lymphangioma in abdominal cavity were retrospectively reviewed. Seven patients displayed chyloperitoneum and chylothorax. Mean time of sirolimus exposure was 19 ± 12.99 months.

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