Abdominal lymphangiomatosis in a 38-year-old female: case report and literature review

Abstract

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.

Figure 1

Chest computed tomography scaning. A: Low density lesion (arrow) occurs along the pulmonary vessels; B: Small effusion (arrow) in the left pleural cavity.

Figure 2

Abdominal computed tomography reveals multiple small cystic lesions (arrow) without enhancement in the abdominal cavity.

Figure 3

Computed tomography enterograph reveals multiple small cystic lesions (arrow) without enhancement in the abdominal cavity. A: Sagittal images; B: Coronal images.

Figure 4

Single balloon enteroscopy reveals duodenal lymphangiectasia with hemorrhage (arrow).

Figure 5

Broken vesica of the gross specimen (arrow).