Lymphangiomatous Lesions of the Gastrointestinal Tract: A Clinicopathologic Study and Comparison Between Adults and Children.

Abstract

Lymphangiomatous lesions involving the gastrointestinal (GI) tract remain incompletely characterized, and their clinical and histopathologic features have not been systematically evaluated. The distinction between a primary lymphatic malformation (lymphangioma) and a dilation of existing lymphatics (lymphangiectasia) is of clinical significance, since lymphangiectasia may occur in the setting of lymphatic obstruction due to an unsampled malignancy. We describe clinical and morphologic features of lymphangiomas of the GI tract in adult and pediatric populations and contrast them with lymphangiectasia. We performed a retrospective review of adult and pediatric lymphangiomas and lymphangiectasia involving the GI tract. Thirty-six cases of lymphangioma and lymphangiectasia were retrieved, and clinical presentation and histologic features were compared. Lymphangiomas had distinct clinical presentations in adults and children, with adult lesions being more frequently asymptomatic and more frequently involving the superficial mucosal layers of the GI tract. Microscopically, lymphangiomas mostly consisted of confluent dilated spaces with a smooth muscle component. This appearance differed from lymphangiectasia, which lacked a complete distinct endothelial or smooth muscle lining and diffusely involved the mucosa and submucosa. Morphologic features of GI tract lymphangiomas can be reliably distinguished from lymphangiectasia by clinical and pathologic characteristics.