RETROPERITONEAL LYMPHANGIOMYOMATOSIS WITH LYMPHEDEMA OF THE LEGS

Retroperitoneal tumor is demonstrated by lymphangiography in a 28 year old woman with lymphedema of both legs. Retroperitoneal tumor was subtotally extirpated, with subsequent subsidence of the swelling of both legs. According to the pathologic examinations of the extirpated specimens, lymphangiomyomatosis was confirmed. This is the fust case reported in the literature of lymphangiomatosis presenting with lymphedema of the leg in the absence of pulmonary involvement, pleural or abdominal effusion.