[A case of primary lymphangiomyomatosis in retroperitoneal space and lungs]

Authors present a rare case of primary lymphangiomyomatosis. Diagnosis was based on histopathological evaluation. Diagnostic difficulties were caused by lack of pathognomonic symptoms in clinical examination, ultrasonography and CT. The patient has been treated with hormonal medicines (Provera, Megace) since 1997. Now she is in a good condition, She hasn't got any symptoms of progression disease.