Lymphangioma is an uncommon benign tumor that develops in the lymphatic system. Abdominal lymphangiomatosis is extremely rare in adult patients, and the clinical symptoms of this condition are complicated and atypical. We report a case of abdominal lymphangiomatosis in a 38-year-old female who presented with intestinal bleeding and protein-losing enteropathy, as well as lesions in the lung and bones. A computed tomography scan revealed multiple small cystic lesions without enhancement. Histological examination revealed microscopic cysts were submucosal, with walls composed of thin fibrous tissue, and D2-40 stained highlight the lining of the lymphatic channels by immunohistochemical method. We make a comparison with the cases reported before, and also discuss the diagnose of diffuse pulmonary lymphangiomatosis and Gorham's disease.
Keywords: Abdominal lymphangiomatosis; Diffuse pulmonary lymphangiomatosis; Gastrointestinal bleeding; Gorham’s disease; Lymphangioma.