Lymphangiomatosis

Lymphangiomatosis is a rare mesenchymal disorder that is characterized by developmental "malformation" of multiple lymphatic channels (usually with dilatation).

Terminology

If lymphatic channels are purely dilated and not malformed the term lymphangiectasia is usually used. If lymphangiomatosis occurs in a singular localized form it is then the term lymphangioma can be used, and if it is widespread throughout the body then generalized lymphatic anomaly can be used.

Pathology

The condition can have various forms depending on organ involvement:

• perirenal/renal lymphangiomatosis
• thoracic lymphangiomatosis
  • pulmonary lymphangiomatosis / pulmonary lymphangiectasia
• abdominal 
  • hepatic lymphangiomatosis
• multisystem 
  • Kaposiform lymphangiomatosis

Associations

• Gorham disease ²