Understanding Lymphangiomatosis

What is lymphangiomatosis?

Lymphangiomatosis is a rare disorder believed to be congenital (something that people are born with) and can affect any of the body's systems except the central nervous system (including the brain). It is a disease of the lymphatic system, which is a network that is linked throughout all areas of the body except the central nervous system.

How does lymphangiomatosis affect the body?

Lymphangiomatosis can occur in every part of the body except the central nervous system and brain because the lymphatic system does not link with these parts of the body.

Parts of the body that can be affected include:

  • Lungs
  • Heart
  • Skeleton
  • Spleen
  • Skin
  • Abdomen
  • Pericardium (the sac around the heart)
  • Kidneys
  • Liver

Lymphangiomatosis can occur in just one part of your body or be spread around different parts of your body.

Doctors cannot gauge how the disease will affect one patient from another, or how quickly it will progress. It seems that lymphangiomatosis usually progresses slowly when it affects the lungs.

Lymphangiomatosis causes tumors to grow wherever it occurs in the body. The tumors are benign (not cancerous). But the great number of tumors that can grow can interfere with your body's normal functions.

Who gets lymphangiomatosis?

Lymphangiomatosis can affect anyone—male, female, any race.

Infants and young children have the highest rate of lymphangiomatosis.

Almost all people with lymphangiomatosis are diagnosed before age 20.

Nobody knows for sure how many people actually have lymphangiomatosis because it is rare and may be often misdiagnosed.

How serious is lymphangiomatosis?

How well people can function and how long they survive with lymphangiomatosis varies. It depends on how severely the disease affects each person's body.

Lymphangiomatosis is most serious for infants whose lungs are affected.

Some people with lymphangiomatosis have survived into adulthood.

There is no proven treatment or cure for lymphangiomatosis.