The lining may be lymph or blood vessel endothelium, but the tissue is derived from the same cells. Nomenclature is confused. Expression of the process can be limited to one focus or can be more widespread. Involvement of the pelvis is more common. The lungs may be involved and the process is a cause of chylous pleural effusions.
Presentation is anytime, but often adolescence.
Massive osteolysis or Gorham's disease can be included in this name. Usually a single bone is involved and replaced by cystic spaces, often without bone reaction. Involvement can begin with erosion of the margin of a bone. Various anatomic expressions of pathology.