Clinical Staging of Hodgkin Lymphoma

The Ann Arbor System (with Cotswolds Modification)

This is the standard staging system for Hodgkin lymphoma (HL), originally developed in 1971 and later modified at Cotswolds (1989). The Lugano Classification (2014) is its current practical update used in most modern protocols.

The Four Stages

Stage	Involvement
I	Single lymph node region or a single extralymphatic site (IE)
II	Two or more lymph node regions on the same side of the diaphragm (II), or limited contiguous extranodal involvement (IIE)
III	Lymph node regions on both sides of the diaphragm (III); may include local extralymphatic extension (IIIE), spleen (IIIS), or both (IIISE)
IV	Diffuse or disseminated involvement of one or more extralymphatic organs (bone marrow, liver, lung, bone) with or without lymph node involvement

The spleen and Waldeyer's ring each count as nodal sites in this system. - Goldman-Cecil Medicine

Suffix Designations

Suffix	Meaning
A	No B symptoms (asymptomatic)
B	Presence of at least one B symptom: unexplained fever >38°C, recurrent night sweats, unexplained weight loss >10% of body weight within 6 months before staging
E	Limited, contiguous extranodal extension that can be included in an irradiation field
S	Splenic involvement
X (older) / Bulky	Any tumor mass with largest diameter ≥10 cm, OR mediastinal mass >1/3 the transthoracic diameter

Disease Classification by Stage

Limited (early) stage: Stages I and II without bulky disease
- In Europe, further subdivided into favorable vs unfavorable limited
Advanced stage: Stage III, Stage IV, or any stage with bulky disease

Lymph Node Regions Used in Staging

Anatomical diagram of lymph node regions for Hodgkin staging

Lymph node regions include: Waldeyer ring, cervical/supraclavicular/occipital/preauricular, mediastinal, hilar, axillary/pectoral, infraclavicular, epitrochlear/brachial, splenic, para-aortic, mesenteric, iliac, inguinal/femoral, and popliteal

Workup Required for Staging (Table 172-2, Goldman-Cecil)

Complete history - B symptoms, other constitutional symptoms
Physical exam - lymphadenopathy, organomegaly
CBC, ESR, LFTs (bilirubin, ALP, LDH), renal function, serum albumin, protein electrophoresis
HIV, Hep B and C serology
Chest X-ray (PA and lateral)
CT scan - neck, thorax, abdomen, pelvis (contrast-enhanced, ≤1 cm slices)
FDG-PET/CT - now considered mandatory; more sensitive/specific than CT alone; replaces the need for bone marrow biopsy in staging

Lugano Classification (2014 Update)

The current standard in most guidelines, based on Ann Arbor with Cotswolds modifications:

Stage	Characteristics
I	One lymph node region or group of adjacent nodes; or single extranodal lesion (IE)
II	≥2 lymph node regions, same side of diaphragm; or limited contiguous extranodal involvement (IIE)
III	Lymph node regions or lymphoid structures on both sides of diaphragm
IV	Non-contiguous extralymphatic involvement
A/B	Absence/presence of B symptoms (for HL only)
E	Extranodal involvement
Bulky	Mass >10 cm or >1/3 of transthoracic diameter; described by size, no longer labeled "X"

(Fishman's Pulmonary Diseases, Table 82-7)

Survival by Stage (Approximate)

Stage	Cure Rate
I-II (limited, non-bulky)	>90-95%
IIIA	30-90%
IIIB	60-70%
IV	50-60%

Key clinical notes:

Stage IV disease is effectively confined to bone marrow, liver, lung, or bone. HL at any other extranodal site should prompt questioning of the diagnosis or a search for HIV infection.
B symptoms are a major prognostic marker - stage IIB behaves more like advanced disease than early disease.
PET/CT is now the gold standard both for initial staging and for interim response assessment during chemotherapy (used to guide consolidation radiotherapy decisions).

Sources: Goldman-Cecil Medicine (Table 172-2, 172-3), Fischer's Mastery of Surgery (Table 188.2), Fishman's Pulmonary Diseases (Table 82-7)

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