Staging and prognosis of Hodgkin lymphoma
- Peter M Mauch, MD
Peter M Mauch, MD
- Professor of Radiation Oncology
- Harvard Medical School
- George P Canellos, MD
George P Canellos, MD
- Editor-in-Chief — Oncology
- Section Editor — Miscellaneous Tumors
- Professor of Medicine
- Harvard Medical School
Once a diagnosis of Hodgkin lymphoma (HL, formerly called Hodgkin's disease) is made, the clinical stage of disease is determined to guide treatment and act as a measure of prognosis. The main goals of staging are to measure the extent of disease and associated prognostic factors, and to define disease manifestations that can be reevaluated during and after treatment to determine the effectiveness of therapy. Staging also serves an important role for the comparison of treatments among studies.
The TNM (tumor node metastasis) staging system that is generally used for solid tumors is not applicable to lymphoma, since it is based upon the concept of a primary tumor and metastasis. Instead, the staging system used for HL is an anatomical classification, which is based upon the concept that HL spreads in a predictable pattern of contiguous disease. HL starts at a single site within the lymphatic system, usually a lymph node, and then progresses to adjacent lymph nodes via lymphatic channels before disseminating to distant nonadjacent sites and organs.
Patients with HL are staged according to the Ann Arbor staging system with Cotswolds modifications [1,2]. This is a four-stage system (stages I to IV). The original Ann Arbor staging system was developed in 1974 and was principally based upon the use of staging laparotomy and lymphangiogram . In 1988, the Cotswold modifications added valuable information regarding the presence of bulky disease and the efficacy of imaging studies for staging (table 1) . In 2014, the Lugano classification proposed further revisions to clarify the role of positron emission tomography (PET) and better define extranodal involvement [3,4].
For treatment purposes, cases of HL are commonly classified as early stage disease (stage I to II) or advanced stage disease (stage III to IV). Patients with early stage HL are then further stratified into favorable and unfavorable subsets.
This topic review will discuss the staging of patients with HL. Other topic reviews discuss the clinical presentation, diagnosis, treatment, and other prognostic measures of these patients. (See "Epidemiology, pathologic features, and diagnosis of classical Hodgkin lymphoma" and "Initial evaluation and diagnosis of classical Hodgkin lymphoma in adults" and "Overview of the treatment of classical Hodgkin lymphoma in adults".)
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- ANN ARBOR STAGING SYSTEM
- Overview of staging notation
- Staging studies
- Determining organ involvement
- - Lymph nodes
- - Spleen
- - Liver
- - Bone marrow biopsy
- - Central nervous system
- - Other sites
- Criteria for B symptoms
- Criteria for bulky disease
- Criteria for extranodal spread
- EXAMPLES OF STAGING
- International Prognostic Score
- Favorable or unfavorable risk early stage disease
- INFORMATION FOR PATIENTS