Extrapulmonary lymphangioleiomyoma presenting as a mesenteric cystic mass

Abstract

Lymphangioleiomyoma (LAM) is a rare benign tumor characterized by a disorganized smooth muscle cell proliferation within the walls of pulmonary, mediastinal, and retroperitoneal lymphatic vessels. The mesenteric LAM is a quite unusual presentation. A 47‐year‐old woman was admitted to our hospital with a 7‐year history of abdominal mass. A computed tomography showed a well marginated mass with soft tissue attenuation. Laparoscopic excision was performed. The specimen revealed a well circumscribed dark cystic mass. The cut surface showed a thin walled cystic tumor with haphazardly distributed trabeculated septa. Microscopically, the tumor showed variable sized ectatic spaces lined by flattened endothelial cells and surrounded by epithelioid or spindle‐shaped cells. The lining cells were positive for D2‐40, CD31, and CD34. The surrounding cells were positive for alpha‐smooth muscle actin and human melanoma black‐45. The final diagnosis was a solitary LAM arising in the mesentery. The patient showed an uneventful postoperative course for 10 months.