Lymphangioleiomyomatosis initially presenting with abdominal pain: a case report

Lymphangioleiomyomatosis (LAM) is a rare idiopathic disease characterized by hamartomatous smooth muscle proliferation of the lymph node, lymphatics, blood vessels as well as airways within the lungs, mediastinum and abdomen. It exclusively affects women during the reproductive period. Though extrapulmonary manifestations have been reported, the initial presentation of LAM as abdominal pain is extremely rare. To our knowledge, there are only three cases with LAM presenting first with abdominal symptoms so far [Chest 106 (1994) 267; Eur J Radiol 14 (1992) 192; Eur J Surg 157 (1991) 36]. We describe a case of LAM suffering from abdominal pain followed by pulmonary symptoms and the diagnosis was not made until pathohistological examination.