Revision 28 for 'Lymphoma (staging)'

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Lymphoma staging

There are a number of lymphoma staging systems for both Hodgkin lymphoma and Non-Hodgkin lymphoma including the Ann Arbor classification, Cotswolds-modified Ann Arbor classification, and the Lugano classification

Staging

Cotswolds-modified Ann Arbor classification

Accurate CT staging has been shown to have a significant impact on treatment and eventual clinical outcome.

  • stage I: one nodal group or lymphoid organ (e.g. spleen or thymus)
    • stage IE: one extranodal site
  • stage II: two or more nodal groups, same side of diaphragm
    • stage IIE: localized extranodal site with stage II criteria, both on the same side of the diaphragm
  • stage III: nodal groups on both sides of the diaphragm
    • stage IIIS(1): with splenic involvement
    • stage IIIE(2): with localized extranodal site
    • stage IIISE: both
  • stage IV: disseminated involvement of one or more extralymphatic organ (e.g. lung, bone) +/- any nodal involvement

Additional staging variables:

  • A: asymptomatic
  • B: presence of B symptoms (fever, night sweats and weight loss)
  • X: bulky nodal disease: nodal mass >1/3 of intrathoracic diameter or 10 cm in dimension
Lugano Classification

Evolution of lymphoma staging and treatment response evaluation criteria:

- 1971: Ann Arbor Staging System;

- 1989: Cotswolds modifications of the Ann Arbor Staging System with introduction of the use of computed tomography (CT);

- 1999: The International Working Group Response Criteria: five clinical response categories based on lesions sizes;

- 2007: The International Harmonization Project Criteria: CT evaluation with additional use of relative qualitative evaluation of 2-deoxy-2-[fluorine-18]fluoro-D-glucose (18FDG) uptake on positron emission CT with 18FDG  (18F-FDG PET/CT);

- 2009: Deauville scale: qualitative evaluation with graduation of 18FDG uptake on 18F-FDG PET/CT with a five-point scale;

- 2014: Lugano Classification.

The Lugano Classification resulted from meetings in 2011 and 2013. The goal of this classification is to simplify and standardize the response assessment and reporting, enabling better understanding and communication among professionals. 

In addition to using CT, classification contemplates the use of 18F-FDG PET/CT for staging and interim treatment response assessment in cases of 18F-FDG-avid lymphoma (Hodgkin and non-Hodgkin subtype diffuse large B-cell). Combined 18F-FDG PET/CT has been found to be more accurate than CT alone for response assessment. Lymphoma types with low or variable FDG uptake should keep being staged with CT. 

Diagnostic contrast-enhanced CT examination should still be included at initial staging for optimal anatomic assessment, which may be completed as part of the 18F-FDG PET/CT. The new criteria for response assessment using CT and 18F-FDG PET/CT are summarized in table 1.

How to make the lesions measurements

- Eligible lesions:

a) lymph nodes: the longest diameter in axial plane should be greater than 1.5 cm;

b) extranodal lesions: the longest diameter in axial plane should be greater than 1.0 cm.

- Lesions chosen should be measurable in two dimensions. In figures 1, 2 and 3 we can see examples how to measure the nodal and extranodal lesions.

- CT (or 18F-FDG PET/CT): tumor burden is to be calculated at baseline staging.

- Choose up to six of the largest nodes, nodal complexes or other lymphoma deposits.

- Measure the longest diameter and shortest diameter of each lesion in axial plane. Multiply the longest diameter and shortest diameter for each lesion to give the “product of the diameters”. Add these to give the “sum of the product of the diameters” (SPD). The SPD calculated at time of staging will serve as the baseline for sequential quantification of tumor burden at interim and end-of-therapy FDG PET/CT.

- Spleen measurement includes the largest transverse diameter and the perpendicular diameter in axial plane, besides the craniocaudal diameter in coronal plane. Splenomegaly is defined as vertical splenic length greater than 13.0 cm (figures 4 and 5).  

Some recommendations of Lugano classification:

- Update of the Ann Arbor classification for anatomic description of disease extent: previously Ann Arbor stage I or II are now categorized as having “limited” disease and previously Ann Arbor stage III or IV are now categorized as having “advanced” disease.

- Update of Cotswolds modifications: as presence of B symptons affects only Hodgkin lymphoma treatment, the modifier “B” should be used in patients with this lymphoma type. The associated “X” modifier is no longer applied in Hodgkin or non-Hodgkin lymphoma; instead, the longest diameter of a mass is simply recorded for staging purposes. 

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