What Is Lymphangiomatosis?

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  • Written By: Jennifer Long
  • Edited By: Allegra J. Lingo
  • Last Modified Date: 01 December 2018
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Lymphangiomatosis (LYMF) is a disease of the lymphatic system. It can affect all areas of the body except the central nervous system or brain. This disease is a rare type of neoplasm and tends to be widespread rather than localized in one area. It is commonly confused with lymphangioleiomyomatosis, which is caused by abnormal smooth muscle growth.

Lymph nodes connect to vessels in every area of the body except the central nervous system and the brain. The lymphatic system is responsible for distributing bodily fluids other than blood throughout the body. A lymphatic disease or other abnormalities can lead to lesions or lymphangiomas, which are neoplasm growths.

Research shows that lymphangiomatosis can be congenital or result in true neoplasms. Congenital types are caused by abnormalities occurring in the lymphatic system from birth. True neoplasms are new tumor growths. The exact cause of this category of rare diseases is not clear.

Most cases of lymphangiomatosis cause scattered lymphangiomas. These neoplasms can be spread out in completely different areas, causing a compression, obstructions, or structural damage because they block the lymphatic vessels. They can also consist of multiple growths that occur in one or more organs. When the growths occur in the organs, damage to the organs can occur and may also lead to additional damage as a result of improper organ function. For example, growths in the lungs can cause oxygen deprivation, which is damaging for other vital organs such as the heart and the brain.


Several factors determine the type of lymphangiomatosis treatment that a patient receives. Not every treatment method will be effective for every patient. It is not uncommon for patients to undergo several types of treatments before a successful one is found. Vascular growth inhibitors can slow the progression for some patients. Chemotherapy is also successful in slowing growth.

The age of the patient and the severity of the disease influence a lymphangiomatosis prognosis. In general, finding an effective treatment quickly can increase the patient’s survival rate. Although most growths are benign, they can pose dangerous risks depending on their location. Whenever possible, surgical removal of growths can increase survival as well. Aggressive growths must be treated aggressively.

Lymphangiomatosis has complications similar to many other neoplasm-related diseases. Regardless of its origin, traditional treatments are not always effective. Doctors must closely monitor growths to find out which modalities work. Younger children have greater odds of successful treatment if the growths are not localized within vital organs.


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