Castleman Disease Stages

After someone is diagnosed with cancer, doctors will try to figure out if it has spread and if so, how far. This process is called staging. The stage of a cancer describes how much cancer is in the body. It helps determine how serious the cancer is and how best to treat it. Doctors also use a cancer's stage when talking about survival statistics.

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 affect 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. Doctors use the results of physical exams and imaging, such as a chest x-ray and either a CT scan or MRI of the chest and abdomen. These are described in Tests for Castleman Disease.

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 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.

The American Cancer Society medical and editorial content team

Our team is made up of doctors and oncology certified nurses with deep knowledge of cancer care as well as journalists, editors, and translators with extensive experience in medical writing.

Fajgenbaum DC, Shilling D. Castleman disease pathogenesis. Hematol Oncol Clin North Am. 2018;32(1):11-21. 

Fajgenbaum DC, van Rhee F, Nabel CS. HHV-8-negative, idiopathic multicentric Castleman disease: Novel insights into biology,  pathogenesis, and therapy. Blood. 2014;123(19):2924-2933. 

Fajgenbaum DC, Uldrick TS, Bagg A, et al.  International, evidence-based consensus diagnostic criteria for HHV-8- negative/ idiopathic multicentric Castleman disease. Blood. 2017;129(12):1646-1657. 

Liebman HA, Tulpule A. Hematologic manifestations of HIV/AIDS. In: Hematology: Basic Principles and Practice. 7th ed. Hoffman R, Benz Jr. E, Silberstein LE, Heslop H, Weitz JI, Anastasi J, Salama ME, Abutalib SA, eds. Philadelphia, PA. Elsevier; 2018: 2262-2277. 

Liu AY, Nabel CS, Finkelman BS, Ruth JR, Kurzrock R, van Rhee F, et al. Idiopathic multicentric Castleman’s disease: A systematic literature
review. Lancet Haematol. 2016;3(4):e163-165.  

Szalat R, Munshi NC. Diagnosis of Castleman disease. Hematol Oncol Clin North Am. 2018;32(1):53-64.

Last Revised: February 2, 2018

American Cancer Society medical information is copyrighted material. For reprint requests, please see our Content Usage Policy.