What is the staging and treatment approach for a patient with Hodgkin lymphoma presenting with an abdominal mass and no other lymph node involvement?

Hodgkin Lymphoma with Isolated Abdominal Mass: Staging and Treatment

A patient with Hodgkin lymphoma presenting with only an abdominal mass and no other lymph node involvement should be staged as early-stage disease (Stage I or II) with extranodal involvement, which classifies them as intermediate-stage unfavorable disease requiring 4 cycles of ABVD followed by 30 Gy involved-field radiotherapy. 1

Staging Classification

The presence of an isolated abdominal mass without other lymph node involvement represents extranodal disease, which is a critical risk factor that automatically upgrades the patient from early favorable to intermediate-stage unfavorable disease, regardless of the Ann Arbor stage. 1

According to the staging criteria:

• Extranodal involvement is specifically listed as a risk factor that moves patients from limited stage to intermediate stage treatment groups 1
• The German Hodgkin Study Group (GHSG) classification explicitly includes extranodal disease as risk factor B, which determines treatment intensity 1
• Even if this is technically Stage I disease (single site), the extranodal location mandates more aggressive therapy 1

Required Staging Workup

Before finalizing treatment, complete the following mandatory evaluations:

• Contrast-enhanced CT scan of neck, chest, and abdomen to confirm no other sites of involvement 2
• PET-CT for accurate initial staging when available 2, 3
• Bone marrow biopsy to exclude marrow involvement 1
• Laboratory assessment: complete blood count, ESR, liver enzymes, alkaline phosphatase, LDH, albumin 1
• Assessment for B symptoms (fever >38°C, drenching night sweats, unexplained weight loss >10% in 6 months) as these affect risk stratification 1, 3

Standard Treatment Approach

The definitive treatment is 4 cycles of ABVD chemotherapy followed by 30 Gy involved-field radiotherapy (IF-RT). 1

This recommendation is based on:

• ESMO guidelines specifically designating extranodal disease as an intermediate-stage risk factor requiring 4 cycles of ABVD plus 30 Gy IF-RT 1
• The GHSG HD11 trial demonstrating tumor control and overall survival exceeding 85-90% at 5 years with this regimen 1
• EORTC trials H7F and H8F confirming that combined modality therapy substantially reduces relapses compared to chemotherapy or radiotherapy alone 1

ABVD Regimen Components:

• Doxorubicin (Adriamycin)
• Bleomycin
• Vinblastine
• Dacarbazine Given every 2 weeks for 4 cycles 1

Radiation Therapy Specifications:

• 30 Gy involved-field radiotherapy to the abdominal mass site 1
• For abdominal wall involvement specifically, ensure adequate radiation field planning with appropriate margins 2
• Some guidelines suggest 36 Gy for stage I-II disease below the diaphragm, though 30 Gy is more commonly used 2

Alternative Intensive Approach (Age-Dependent)

For patients under 60 years old who are eligible for more intensive treatment, consider:

• 2 cycles of BEACOPPescalated followed by 2 cycles of ABVD and 30 Gy IF-RT 1
• This approach showed superior 4-year freedom from treatment failure compared to standard ABVD in the GHSG HD14 trial 1
• However, long-term toxicity data (especially infertility) are lacking, making this a more controversial choice 1

Critical caveat: BEACOPP should never be used in patients over 60 years due to significantly increased toxicity in this age group 1

Response-Adapted Strategy

Modern practice incorporates interim PET-CT assessment:

• Perform PET-CT after 2 cycles of chemotherapy to assess early response 2, 3
• Deauville score 1-3 indicates good response; continue planned therapy 2
• Deauville score 4-5 indicates poor response; consider treatment intensification or clinical trial enrollment 2
• This approach is increasingly used but cannot yet be considered standard outside clinical trials as randomized data are still maturing 1

Critical Treatment Modifications

Age-Related Adjustments:

• Patients over 60 years: Use standard ABVD only; avoid BEACOPP entirely 1
• Consider discontinuing bleomycin after cycle 2 in older patients due to increased pulmonary toxicity risk 2

Fertility Considerations:

• Offer fertility preservation counseling before treatment initiation to all patients of reproductive age 2, 3
• This is particularly important as ABVD has lower gonadotoxicity than BEACOPP, but risks still exist 3

Cardiac Monitoring:

• Baseline echocardiogram or MUGA scan before anthracycline-containing regimens 2, 4
• Monitor cardiac function during treatment given doxorubicin cardiotoxicity 2, 4

Post-Treatment Surveillance

After completing therapy:

• PET-CT at treatment completion to confirm complete remission 2, 3
• Negative PET-CT after chemotherapy may allow omission of radiotherapy in selected patients, though this remains investigational 1
• Long-term follow-up for late effects including secondary malignancies and cardiovascular disease 3

Common Pitfalls to Avoid

1. Do not treat as early favorable disease simply because it appears to be Stage I—extranodal involvement mandates intermediate-stage therapy 1

2. Do not omit radiotherapy in this setting outside of clinical trials, as combined modality therapy remains standard 1

3. Do not use chemotherapy-only approaches (4-6 cycles ABVD without radiation) as standard, since prospective randomized data supporting this are very limited for intermediate-stage disease 1

4. Avoid exploratory laparotomy and splenectomy—these are no longer recommended for staging 1

Prognosis

With appropriate treatment, patients with intermediate-stage Hodgkin lymphoma achieve:

• Tumor control rates of 85-90% at 5 years 1
• Overall survival exceeding 90% at 5 years 1
• The presence of extranodal disease as a single risk factor has a favorable prognosis when treated appropriately 1