Lymphangioleiomyomatosis [
LAM] is a rare
lung disease affecting women and characterized by abnormal smooth muscle cells (
LAM cells) proliferation along lung and lymphatic channels. The frequent occurrence of extrapulmonary
LAM [e-
LAM] has been reported as abdomen pelvic lymph nodes involvement,
angiomyolipomas,
lymphangioleiomyomas or
lymphangiomas in
LAM patients. An extrapulmonary manifestation as the initial
LAM presentation preceding pulmonary disorders and as asymptomatic extrapulmonary
LAM lesions are unusual. We report two women presented with asymptomatic retroperitoneal cystic masses accidentally found on ultrasound examination. The tumours were surgically removed and diagnosed as: 1-malignant
mesothelioma and 2-tymphangiomyoma. The microscopical sections were reviewed and re-diagnosed as e-
LAM at advanced pulmonary
LAM development. Mesotheliosis present in e-
LAM morphology is unique and was misleading for
malignancy diagnosis. The second case illustrates the
hormone dependent growth of
lymphangiomyoma and
LAM development in young women. It is difficult to prove the presence of pulmonary
LAM at the time of tumours excision but both cases demonstrate importance of appropriate
LAM diagnosis and being aware of such diagnosis in cases presenting with extrapulmonary extension of the disease.