ABSTRACT
Primary lymphedema may be classified as a “truncular” lymphatic malformation based on the Hamburg classification that develops during the late stages of lymphangiogenesis, ultimately resulting in hypoplasia, hyperplasia, or aplasia of lymph vessels. Lymphangiomas are an “extra-truncular” type of lymphatic malformation developed during the early stages of lymphangiogenesis. Lymphangiomas, also termed/defined as lymphatic malformation by International Society for the Study of Vascular Anomalies (ISSVA) classification, are benign congenital vascular malformations of the lymphatic system that can occur anywhere on the skin and mucous membranes. Lymphangiomas can be categorized as deep or superficial, or further as congenital or acquired based on an old concept. And they also can be central conducting lymphatic anomalies.
