Primary abdominal lymphangioleiomyomatosis: report of a case.

Lymphangioleiomyomatosis is an uncommon progressive disease characterized by hamartomatous smooth muscle proliferation of the airways within the lungs as well as the lymph nodes, lymphatics, and blood vessels of the lungs, mediastinum, and abdomen. The most common manifestations of lymphangioleiomyomatosis are pulmonary symptoms. Primary abdominal lymphangioleiomyomatosis without any pathological changes in the respiratory system is extremely unusual. We report a case of primary abdominal lymphangioleiomyomatosis located between the left hepatic and gastric antrum of a 29-year-old woman. The patient had no typical symptoms of lymphangioleiomyomatosis (dyspnea, pneumothorax) or abdominal pain. All physical examination findings were normal. Laboratory test results, including routine blood examination, liver and kidney function, tumor markers, blood coagulation function, and urine and stool examinations, were all normal. She found abdominal cyst in an annual medical examination by ultrasonography and confirmed by computed tomography. For a clear diagnosis, a laparoscopic abdominal mass resection was performed. The postoperative pathohistological examination findings allowed for the definitive diagnosis. This case report may advance the understanding of primary peritoneal lymphatic leiomyoma and reduce the number of mistakenly diagnosed patients.