Case of lymphangiomyomatosis of the kidney and retroperitoneum

: We report a 44-year-old female with lymphangiomyomatosis (LAM) of the kidney and retroperitoneum. Abdominal ultrasonography revealed a right kidney tumor, and she was referred to our department. Computed tomography (CT) revealed a para-aortic phyma in addition to the tumor. We performed retroperitoneal tumorectomy and partial resection of the right kidney via laparotomy. Pathological findings suggested LAM. LAM usually induces pulmonary lesions and its prognosis is relatively unfavorable. Female hormones are considered to be involved in the aggravation of LAM. The lesion may not have reached the lung in this postmenopausal woman because of the absence of these hormones.