Lymphangiomatosis and the Lymph System

Lymphangiomatosis is a disease in which many tumors (lymphangiomas) or cysts grow in the lymph system of the body. Although these tumors are not cancerous, they invade the body tissues and cause pain, difficulty breathing, and a variety of other symptoms depending on where they occur. Lymphangiomas may grow in the bones, connective tissue, and organs of the body, and they may obstruct, compress, or destroy tissues. What causes lymphangiomatosis is not yet known.

Lymphangiomatosis is most often diagnosed in infants and young children but may occur at any age. It affects both males and females of all ethnic backgrounds. Because lymphangiomatosis is rare and difficult to diagnose correctly, it is not known exactly how many people are affected by it throughout the world.

Symptoms

The symptoms of lymphangiomatosis depend on where the tumors are growing in the body.

• Lungs – may cause a chronic cough, wheezing, shortness of breath, a collection of fluid around the lungs
• Heart – rapid heartbeat, chest pain
• Stomach, intestines – abdominal pain, nausea, vomiting, diarrhea
• Kidneys – pain, increased blood pressure
• Bones – pain, weakening of the bone leading to fracture, pinched nerves in the spine

Over time, one or more bones may be replaced by lymphangioma tissue, which is known as Gorham’s disease. Lymphangiomas do not grow in the brain because the lymph system does not extend into it.

Diagnosis

Diagnosis of lymphangiomatosis is based on the symptoms and the presence of many lymphangiomas in the body. CT (computed tomography) scan and magnetic resonance imaging (MRI) is used to evaluate the disease. A tissue sample (biopsy) is taken to help confirm that the tumors are lymphangiomas.

Treatment

Lymphangiomatosis slowly worsens over time. As the tumors grow, they may cause serious symptoms, some of which may be life-threatening, such as causing fluid to collect around the lungs that restricts breathing. Surgical removal of tumors is possible, but some tumors may be difficult to completely remove if they have spread out. A medication called Intron A (interferon alfa), chemotherapy, or radiation therapy may be helpful in treating the disease. Other treatments or surgeries may be helpful in relieving some of the symptoms or problems caused by lymphangiomatosis. For example, a tube may be inserted into the chest (thoracentesis) to drain off accumulated fluid, or surgery may be done on the spine to relieve pinched nerves.