Castleman Disease

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Early Detection, Diagnosis, and Staging TOPICS

How is Castleman disease staged?

When talking about cancer, the stage is a description of how far it has spread. The stage helps doctors determine the best treatment and the likely outlook (prognosis) for the patient. Most cancers have a formal staging system that lets doctors sum up the extent of the cancer.

Since Castleman disease (CD) is not a cancer, it doesn’t have a formal staging system. Instead, doctors use other important pieces of information to help decide on the best treatment and to give them an idea of how well a patient might do.

The most important factor when deciding on treatment is whether the CD is localized/unicentric or multicentric. Localized/unicentric CD affects only a single lymph node (or lymph node group). The multicentric type affects 2 or more groups of lymph nodes in different parts of the body. It may also involve internal organs like the spleen or liver. Tests are done to see what lymph nodes and organs are affected to learn which type of CD a patient has. These tests often include some of the imaging tests mentioned earlier, such as a chest x-ray and either a CT scan or MRI of the chest and abdomen.

Another factor is the microscopic subtype of the CD, which is a description of the patterns of cells seen under the microscope in the biopsy sample. These subtypes are described in the section, “What is Castleman disease?

A third important factor is whether or not the patient is infected with the human immunodeficiency virus (HIV), the virus that causes AIDS. Just about all people infected with HIV who develop CD will have the multicentric form of the disease. These patients are among the hardest to treat.

Outlook (prognosis) for people with Castleman disease

Some people may want to know statistics on the outlook for those in similar situations, while others may not find the numbers helpful, or may even not want to know them. Whether or not you want to read about the statistics below for CD is up to you.

It is hard to get accurate numbers on the outlook for people with CD because it is rare. Most statistics on the disease come from small numbers of patients who were treated at a single center or hospital. These numbers might not accurately reflect the outcomes for all people with CD.

The numbers below come from a study of nearly 200 people treated for Castleman disease in many different centers. The study divided people into groups based on whether the CD was unicentric or multicentric, its microscopic subtype, and whether the patient was infected with HIV (HIV+) or not infected (HIV-).

The 3-year disease-free survival (DFS) rate refers to the percentage of patients who were still alive and had no signs of CD at least 3 years after it was diagnosed. Of course, many people went much longer than 3 years without any signs of CD (and many were likely cured).

Type of Castleman Disease

3-Year Disease-Free Survival

Unicentric, hyaline vascular, HIV-


Unicentric, plasma cell or mixed, HIV-, OR
Multicentric, hyaline vascular, HIV-


Multicentric, plasma cell, HIV-


HIV+ (multicentric)


Even when taking the factors above into account, disease-free survival rates are at best rough estimates. Your doctor can tell you how well these numbers may apply to you, as he or she is familiar with your particular situation.

Last Medical Review: 06/11/2012
Last Revised: 06/11/2012