Embryonic Development and Malformation of Lymphatic Vessels

  1. Derek J. Chadwick Organizer and
  2. Jamie Goode
  1. Jörg Wilting1,
  2. Kerstin Buttler1,
  3. Jochen Rössler2,
  4. Susanne Norgall3,
  5. Lothar Schweigerer1,
  6. Herbert A. Weich3 and
  7. Maria Papoutsi1

Published Online: 11 SEP 2007

DOI: 10.1002/9780470319413.ch17

Vascular Development: Novartis Foundation Symposium 283

Vascular Development: Novartis Foundation Symposium 283

How to Cite

Wilting, J., Buttler, K., Rössler, J., Norgall, S., Schweigerer, L., Weich, H. A. and Papoutsi, M. (2007) Embryonic Development and Malformation of Lymphatic Vessels, in Vascular Development: Novartis Foundation Symposium 283 (eds D. J. Chadwick and J. Goode), John Wiley & Sons, Ltd, Chichester, UK. doi: 10.1002/9780470319413.ch17

Author Information

  1. 1

    Zentrum für Kinderheilkunde und Jugendmedizin, Pädiatrie I, Forschungslabor, Georg-August-Universität Göttingen, Robert-Koch-Strasse 40, 37075 Göttingen, Germany

  2. 2

    Department of Pediatrics, Albert- Ludwigs-Universität, Freiburg, Germany

  3. 3

    Department Gene Regulation and Differentiation, German Research Centre for Biotechnology, Braunschweig, Germany

Publication History

  1. Published Online: 11 SEP 2007
  2. Published Print: 20 JUL 2007

Book Series:

  1. Novartis Foundation Symposia

Book Series Editors:

  1. Novartis Foundation

ISBN Information

Print ISBN: 9780470034286

Online ISBN: 9780470319413



  • lymphatic endothelial cells (LECs);
  • VEGFR3 and lymphangiomas;
  • tumour-induced lymphangiogenesis;
  • blood vascular endothelial cells (BECs);
  • lymphatic vessel development and malformation


In the human, malformations of lymphatic vessels can be observed as lymphangiectasia, lymphangioma and lymphangiomatosis, with a prevalence of 1.2–2.8%. Their aetiology is unknown and a causal therapy does not exist. We investigated the origin of lymphatic endothelial cells (LECs) in avian and murine embryos, and compared the molecular profile of LECs from normal and malformed lymphatics of children. In avian embryos, Prox1+ lymphangioblasts are located in the confluence of the cranial and caudal cardinal veins, where the jugular lymph sac (JLS) forms. Cell lineage studies show that the JLS is of venous origin. In contrast, the lymphatics of the dermis are derived from mesenchymal lymphangioblasts located in the dermatomes, suggesting a dual origin of LECs in avian embryos. The same may hold true for murine embryos, where Lyve1+ LEC precursors are found in the cardinal veins, and in the mesenchyme. The mesenchymal cells express the pan-leukocyte marker CD45, indicating a cell type with lymphendothelial and leukocyte characteristics. In the human, such cells might give rise to Kaposi's sarcoma. Microarray analyses of LECs from lymphangiomas of children show a large number of regulated genes, such as VEGFR3. Our studies show that lymphvasculogenesis and lymphangiogenesis occur simultaneously in the embryo, and suggest a function for VEGFR3 in lymphangiomas.