Extrapulmonary lymphangioleiomyomatosis with retroperitoneal lymph node involvement

Lymphangioleiomyomatosis (LAM) is a rare disease of unknown etiology that occurs almost exclusively in women, primarily of reproductive age. This disease is characterized by smooth muscle cell proliferation, most commonly in the lungs. However, cases of extrapulmonary LAM have been reported, such as in the lymph nodes of mediastinum, abdominal cavity, and retroperitoneum. The clinical manifestations of pelvic lymph node LAM are subtle manifesting as symptoms of compression due to a large tumor mass but more often diagnosed incidentally in lymph node specimens removed during gynecologic oncology surgeries. This article provides literature data and our own observation of lymph node LAM in a patient with metastatic endometrial cancer.