Laboratory models for the investigation of lymphangiomatosis

Microvasc Res. 2014 Nov;96:64-7. doi: 10.1016/j.mvr.2014.08.007. Epub 2014 Aug 28.


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.

Keywords: Gorham–Stout disease; Lymphangiectasia; Lymphangioma; Lymphatic malformation; VEGF-C; Wnt5A.

Publication types

  • Review

MeSH terms

  • Animals
  • Disease Models, Animal
  • Endothelial Cells / cytology
  • Humans
  • Lung Diseases / congenital*
  • Lung Diseases / diagnosis
  • Lung Diseases / pathology
  • Lymphangiectasis / congenital*
  • Lymphangiectasis / diagnosis
  • Lymphangiectasis / pathology
  • Lymphatic Vessels / pathology*
  • Mice
  • Mice, Inbred C57BL
  • Mice, Knockout
  • Osteolysis, Essential / pathology
  • Proto-Oncogene Proteins / metabolism
  • Vascular Endothelial Growth Factor A / metabolism
  • Wnt Proteins / genetics
  • Wnt Proteins / metabolism
  • Wnt-5a Protein


  • Proto-Oncogene Proteins
  • Vascular Endothelial Growth Factor A
  • WNT5A protein, human
  • Wnt Proteins
  • Wnt-5a Protein
  • Wnt5a protein, mouse

Supplementary concepts

  • Lymphangiectasia, pulmonary, congenital