Lymphangioleiomyomatosis suspected to be a gynecologic disease

Lymphangioleiomyomatosis (LAM) is a rare, systemic disorder that predominantly affects women. Although patients with LAM mostly present with pulmonary symptoms, some patients may present initially with extrapulmonary symptoms. We present a case of a 30-year-old Japanese female with abdominal pain during menstrual periods was suspected of having ovarian cancer due to exaggerated ascites observed at a local clinic. Subsequently, she was transferred to our hospital for further investigations, and was diagnosed with LAM. Three years after diagnosis, she had a girl by cesarean section to avoid the progression of pulmonary LAM by vaginal delivery. The patient is undergoing follow-up treatment with the administration of gonadotropin-releasing hormone-analog. Though LAM is rare, gynecologists should know about it because it may occur with gynecological symptoms in young women.