Retroperitoneal Lymphangioleiomyomatosis without Pulmonary Feature

ABSTRACT

Lymphangioleiomyomatosis (LAM) is discovered mainly in the pulmonary area, and the typical presenting symptoms include progressive dyspnea, pneumothorax, and chylous pleural effusion. An initial presentation of LAM with extrapulmonary symptoms is extremely rare. Most reports and reviews have concentrated on the pulmonary symptoms and radiological image of LAM. Some of reports focused on the abdominal abnormalities or retroperitoneal abnormalities in addition to pulmonary features. However, LAM without pulmonary features is extremely rare. We encountered a 36-year-old woman with a retroperitoneal mass measuring 7 cm in the anterior aspect of the right adrenal gland. The patient did not have any pulmonary symptoms or radiological abnormalities, and underwent a laparoscopic excision. The tumor was diagnosed histologically as a lymphangioleiomyomatosis. LAM without pulmonary feature is unusual case. We report this case with a review of the relevant literature.