Laboratory models for the investigation of lymphangiomatosis

• Lymphangiomatosis is an uncommon proliferative disorder of the lymphatic vasculature.
• The etiology remains poorly understood.
• The disease pathology is heterogeneous.
• Several useful model systems are described.
• Future deductive investigation of therapeutics will require more robust suitable animal models.

Lymphangiomatosis is an uncommon proliferative disorder of the lymphatic vasculature whose etiology remains poorly understood. The lymphangiomatosis spectrum encompasses a remarkable heterogeneity in its potential presentation, including micro- and macrocystic isolated lymphatic malformations, thoracic and intraabdominal diffuse lymphangiomatosis, and osseous and soft-tissue presentations known as Gorham–Stout disease. Recent therapeutic advances are empirical in nature or, at best, inferential, reflecting the scanty availability of laboratory-based model systems for the mechanistic study of this disease. Several promising model systems are reviewed here. The laboratory investigation of lymphangiomatosis will likely continue to benefit from the remarkable growth of insights into the mechanisms of lymphangiogenesis and vascular development.