Can you tell me what is Castleman disease and what causes it?

Castleman disease is a benign type of tumor formed by plasma cells in the lymph tissue. It was named for Dr. Castleman who first reported it in 1956. It is fairly rare and usually shows up the in the front part of the chest under the breast bone. This area is called the mediastinum.

It can appear anywhere in the body where lymph tissue (nodes) is abundant. This includes the under arm (axilla) area, abdomen, and neck. In rare cases, the nervous system has been involved. This means the tumor grows inside the brain (intracranial). Even more rare are cases involving the spine.

It's not clear what causes these benign tumors to grow. Theories today include infection, inflammation, and an autoimmune process. Patients present with symptoms of fever, weight loss, anemia, and an elevated sed rate. A high sed rate suggests infection or inflammation.

Treatment is surgical to remove the tumor. Sometimes radiation therapy or chemotherapy is used as well. There aren't enough cases of Castleman disease to compare and find out which treatment works best.

Most patients do quite well after treatment. The tumor does not seem to grow back. Only in rare cases has death been reported as a result of this condition. It may depend on the location of the tumor.

Intracranial tumors can cause seizures and edema (swelling). Older adults may develop more problems and have a lower survival rate.