Surgical management of solitary extrapulmonary lymphangioleiomyomatosis in the mesentry: a case report

Abstract

Pulmonary lymphangioleiomyomatosis (LAM) is a rare condition characterised by infiltration of the lungs with abnormal smooth muscles and cystic lesions. A rarer form of the condition is extrapulmonary LAM (E-LAM) where the same cystic lesions are found in various organs throughout the body resulting in a variety of symptoms. Given the rarity of E-LAM and the difficulty in diagnosing it, there is little evidence to guide its management both surgically and medically. We describe a case of a 22-year-old female with a nine-month history of abdominal pain found to have a large mesenteric mass during laparoscopy for suspected ovarian torsion. She underwent a laparotomy to exteriorise the mass and dissect it off the mesentery. The mass was removed without compromise to the bowel or mesentery. The patient recovered well without symptoms and was discharged without complications. Histopathology of the mass revealed it to be E-LAM. This case is the first to our knowledge that demonstrates the successful removal of a solitary E-LAM from the mesentery with minimal adverse outcomes and symptomatic relief.