Chapter 6 - Defective development of the peripheral lymphatic system: Lymphatic malformations

Abstract

Lymphatic malformations (LMs) are one grouping of numerous forms of congenital vascular malformations (CVMs), affecting only the lymphatic system as the outcome of defective development during various stages of lymphangiogenesis. LM is caused by a developmental arrest in the “earlier” stages of lymphangiogenesis and is classified to the “extratruncular” lesion (also known as “lymphangioma”), since it maintains “evolutional potential” and a unique embryonic characteristic that originates from the mesenchymal cells. LM caused by the lesion/defective development during the “later” stage of lymphangiogenesis is defined as “truncular” lesion (also known as “primary lymphedema”), with no such evolutional potential to grow (cf. extratruncular).