Lymphangioleiomyomatosis and gorham-stout disease: Primary or secondary disorders of the lymphatic system?

Lymphangioleimyomatosis and Gorham-Stout disease, rare disorders which share features of proliferating lymphatic vessels, predominantly in the lung in the former and bone in the latter, commonly manifest as progressive lung disease often associated with life-threatening complications of chylous reflux from central lymphatic obstruction/leakage. This Lymphspiration-Editorial proposes that, rather than a secondary complication, lymphatic obstruction/dysfunction is a much earlier or even the primary event in the pathogenesis of both disorders and that lymphostasis drives the cellular proliferative response in both lung and bone and even distant sites.