Ann Arbor staging system

Staging of Hodgkin and non-Hodgkin lymphomas

1. Determine stage according to sites involved by lymphoma

IInvolvement of a single lymph node region* or lymphoid structure
IIInvolvement of two or more lymph node regions,* or localized involvement of one extranodal site and one or lymph node regions, all on the same side of the diaphragm
IIIInvolvement of lymph node regions* or structures on both sides of the diaphragm
IVDiffuse or disseminated involvement of one or more extralymphatic organs, OR isolated extralymphatic organ involvement without adjacent regional lymph node involvement, but with disease in distant site(s), OR any involvement of the liver, bone marrow, pleura or CSF
*Lymph node regions include: right cervical (including cervical, supraclavicular, occipital, and preauricular lymph nodes), left cervical, right axillary, left axillary, right infraclavicular, left infraclavicular, mediastinal, hilar, periaortic, mesentery, right pelvic, left pelvic, right inguinal femoral, and left inguinal femoral

Adapted from Carbone et al, 19711 and Lister et al, 1989.2

2. Add relevant suffixes

AAbsence of constitutional symptoms
BConstitutional symptoms: fever (> 38°C), drenching sweats, weight loss (10% body weight over 6 months)
EInvolvement of a single, extranodal site contiguous or proximal to a known nodal site (stages I to III only; additional extranodal involvement is stage IV)
SSplenic involvement
XBulky disease, defined as one or more site of disease of > 10 cm diameter, or mediastinal widening to > 1/3 of the chest width on chest X-ray
Other provisions include designation of involved extranodal site(s): M = marrow, L = lung, H = liver, P = pleura, O = bone, D = skin and subcutaneous tissue; and number of sites of disease (e.g. II2, II4)

Adapted from Carbone et al, 19711 and Lister et al, 1989.2

The original Ann Arbor staging system was published in 1971.1 The subsequent ‘Cotswold modifications’ of 1989 extended the definitions of stage IV disease, and added the suffix ‘X’ for bulky disease.2 Both systems were developed for the staging of Hodgkin lymphoma, but are now also widely used to stage non-Hodgkin lymphomas.

The following are classified as stage IV disease (rather than stage IE – IIIE):

  • Any bone marrow, liver, pleural or CSF involvement
  • Diffuse or disseminated extranodal disease
  • Isolated extranodal involvement (as opposed to direct extension from nodal disease), together with distant disease

Some provisions of the Ann Arbor staging system and Cotswold modifications, such as the individual designation of extranodal sites and separate reporting of clinical and pathologic stages, are no longer routinely employed. The more recent Lugano Classification staging system removes these features entirely.