Limited Stage
The Lugano system distinguishes early, limited forms of the disease where the process is localized:
- Stage I: Involvement of a single lymph node region only (including the spleen or Waldeyer's ring).
- Stage IE: Single extranodal lesion site without nodal involvement. For Hodgkin lymphoma (HL), this is an extremely rare scenario.
- Stage II: Two or more lymph node regions are affected, but they are located strictly on the same side of the diaphragm.
- Stage IIE: Contiguous spread of the tumor from a lymph node into adjacent extranodal tissue. Other nodes on the same side of the diaphragm may be involved.
- Stage II massive (bulky): Status depends on the size of the largest tumor conglomerate. For HL and diffuse large B-cell lymphoma (DLBCL), the mass criterion is a size greater than 10 cm (or greater than one-third of the chest diameter on CT for HL). For follicular lymphoma (FL), the threshold is lowered to 6 cm. The historical "X" index is no longer used.
Advanced Stage
These stages indicate generalization of the pathological process:
- Stage III: Lymph node regions are affected on both sides of the diaphragm. This stage also includes involvement of supradiaphragmatic nodes combined with splenic involvement. Important: the IIIE and IIIS indices have been excluded from the modern classification.
- Stage IV: Disseminated involvement of one or more extranodal organs (with or without nodal involvement). This also includes non-contiguous extranodal spread in stage II. Any involvement of the liver, lungs, bone marrow, or central nervous system is regarded as stage IV (with the exception of direct contiguous extension in stage IIE).
Principles of diagnostic imaging
The choice of imaging depends on the ability of the tumor tissue to uptake fluorodeoxyglucose (FDG):
- FDG-PET/CT: The gold standard for FDG-positive lymphomas. It allows distinguishing a tumor from reactive hyperplasia and detecting pathology in normal-sized nodes. Any abnormal tracer uptake is considered pathological. The use of PET/CT leads to upstaging (upstaging) in 30% of patients.
- Contrast-enhanced CT: The standard for FDG-negative forms. Evaluation is based on size: the upper limit of normal for the short axis is 10 mm. Some nodes (in the presacral region or splenic hilum) should normally not be visualized at all.
- MRI: Comparable to CT in accuracy, but eliminates ionizing radiation, which is critical for young patients. Diffusion-weighted imaging (DWI) is actively being implemented.
- Ultrasound: Not suitable for systemic staging; used only for biopsy guidance or evaluation of superficial structures.
Prognostic indices and management
Treatment approaches vary radically depending on the tumor type:
- Hodgkin lymphoma: Treatment is administered with curative intent, with early-stage survival exceeding 90%. The baseline regimen is ABVD with interim PET monitoring. Currently, physicians aim to minimize radiation therapy in young patients due to the risk of secondary cancers and coronary artery disease. For relapses, the targeted drug brentuximab vedotin is prescribed, and autologous stem cell transplantation (ASCT) is performed.
- Non-Hodgkin lymphoma (NHL): The main factor is the histological subtype, not the stage. Prognosis is assessed using the IPI index (age > 60 years, elevated LDH, stage III-IV, >1 extranodal site, poor performance status) and FL-IPI. For asymptomatic FL, a "watch and wait" strategy is justified. For aggressive forms (DLBCL), therapy with the R-CHOP regimen is initiated immediately.
The secret of stages: I and II are always on the same side of the diaphragm, III is on both sides, and IV is dissemination to internal organs (liver, lungs, bone marrow) or the CNS.
Class and exam questions
In which cases can a bone marrow biopsy be omitted?
In routine FDG-avid non-Hodgkin lymphomas, if PET/CT has already reliably demonstrated signs of bone marrow involvement, an additional trephine biopsy is not required.
How to differentiate involved nodes in Hodgkin versus non-Hodgkin lymphomas?
In non-Hodgkin lymphomas, nodes are typically larger and frequently combined with extranodal lesions. In Hodgkin lymphoma, lymph node involvement is usually the sole manifestation.
Does heterogeneous nodal enhancement on CT signify a poor prognosis?
Yes, heterogeneous contrast enhancement on CT or an inhomogeneous signal on T2-weighted MRI images within large tumor masses are associated with a poorer prognosis.
What else to study on this topic
- Criteria of the IPI prognostic index and FLIPI 2 modifications
- Differences between mantle field radiation therapy and modern irradiation protocols
- Algorithm for chemotherapy escalation to the BEACOPP regimen
- Semiotics of lymphomas on magnetic resonance imaging scans
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